Materiały źródłowe

• #1 Hereditary spastic paraplegia (HSP) | Paris Brain Institute

  https://parisbraininstitute.org/disease-files/hereditary-spastic-paraplegia-hsp

  Hereditary spastic paraplegia (HSP), also known as Strmpell-Lorrain disease, is a highly heterogeneous group of diseases. […] The main symptom of the disease is stiffness (spasticity) and weakness of the lower limbs, leading to severe walking difficulties due to damage to the motor neurons. […] These diseases are said to be chronic, progressing at different rates depending on the patient, with some patients becoming totally unable to walk and others showing very few symptoms several years after diagnosis. […] In the pure forms of the disease, which are the most common forms, symptoms include stiffness, spasticity and weakness of the lower limbs, leading to severe walking difficulties. […] Spastic paraplegia is diagnosed on the basis of a clinical examination, a study of the family history and a genetic analysis to find a causative mutation. […] There is currently no cure for these conditions, but treating symptoms such as spasticity can bring relief to sufferers. Physiotherapy, occupational therapy, balneotherapy and treatments based on muscle relaxants or botulinum toxin can help improve motor skills.

• #1 Hereditary spastic paraplegia – UpToDate

  https://www.uptodate.com/contents/hereditary-spastic-paraplegia

  Hereditary spastic paraplegia (HSP) refers to a group of familial diseases that are characterized by progressive degeneration of the corticospinal tracts. Clinically, they present with lower limb spasticity and weakness. […] HSPs are clinically differentiated into „pure” forms if spastic paraplegia with bladder involvement is the only clinical finding, and „complicated” (or complex) forms if there are additional neurologic or systemic abnormalities. […] The genetic classification of HSP is based upon mode of inheritance, chromosomal locus, and causative mutation (if known). Hereditary spastic paraplegias include autosomal dominant, autosomal recessive, and X-linked forms. […] The correlation of clinical classification (pure or complicated) with genetic classification (SPG type) is imperfect, and some genetic types of HSP are associated with both pure and complicated phenotypes.

• #1 Hereditary Spastic Paraplegia | National Institute of Neurological Disorders and Stroke

  https://www.ninds.nih.gov/health-information/disorders/hereditary-spastic-paraplegia

  Currently, no treatments can prevent, slow, or reverse HSP. However, treatments are available to help manage symptoms. For example, muscle relaxers may help reduce stiffness in the legs. Some people benefit from surgery to loosen tight muscles. Assistive devices like braces, walkers, or wheelchairs can also improve mobility. Regular physical therapy can help people with HSP maintain muscle strength and flexibility. […] The outlook for people with HSP varies. Some people experience severe disability, while others have only mild symptoms. Most people with HSP have a normal life expectancy.

• #1 Paraplegia: Nursing Diagnoses & Care Plans | NurseTogether

  https://www.nursetogether.com/paraplegia-nursing-diagnosis-care-plan/

  Paraplegia is characterized by paralysis and loss of sensation in the lower extremities that occurs after a spinal cord injury. With paraplegia, autonomic dysreflexia can occur as an overreaction of the patients autonomic nervous system from noxious stimuli, resulting in potentially life-threatening hypertension. […] The management of paraplegia often focuses on preventing complications and further injuries. Immediate medical treatment can prevent partial paraplegia from becoming complete paraplegia. Physical and rehabilitation therapy is vital to strengthening muscles and alleviating pain. The nurse can support and encourage lifestyle modifications to empower patients to live actively and productively. […] Once the nurse identifies nursing diagnoses for paraplegia, nursing care plans help prioritize assessments and interventions for both short and long-term goals of care. […] Paraplegic patients have a higher risk for injuries because of the loss of function and sensation. […] Rehabilitation programs for paraplegic patients are available to provide support, maintain muscle strength and mass, and teach techniques to maneuver and perform tasks safely and independently.

• #1 Presentation: Patient with suspected hereditary spastic paraplegia — In the Clinic

  https://www.genomicseducation.hee.nhs.uk/genotes/in-the-clinic/presentation-patient-with-suspected-hereditary-spastic-paraplegia/

  Hereditary spastic paraplegia is a rare genetic condition that causes progressive limb stiffness and weakness. […] Hereditary spastic paraplegia (also known as hereditary spastic paraparesis) is a diagnosis of exclusion, and all possible acquired causes of the patients paraplegia should be eliminated prior to genomic testing. […] Genomic testing should be considered in those with unexplained spastic paraplegia with or without a family history. […] A genetic diagnosis may have implications for other family members, and can be particularly relevant during a pregnancy. […] For hereditary spastic paraplegia there are two panels, and the choice should depend on the age of onset in your patient. […] Adult onset hereditary spastic paraplegia. This panel investigates single-gene causes of hereditary spastic paraplegia where onset is in adulthood. […] Childhood onset hereditary spastic paraplegia. This indication investigates single-gene causes of hereditary spastic paraplegia where onset is in childhood. […] The above tests are DNA-based, and an EDTA sample (typically a purple-topped tube) is required.

• #1 Bladder dysfunction in hereditary spastic paraplegia: a clinical and urodynamic evaluation | Spinal Cord

  https://www.nature.com/articles/sc2011193

  Hereditary spastic paraplegia (HSP) is a degenerative central nervous system disorder characterized by progressive spasticity and hyperreflexia of the lower limbs. Often, patients with HSP experience symptoms of voiding dysfunction. […] The present study characterizes lower urinary tract dysfunction in a large series of patients. […] Neurogenic lower urinary tract impairments are well recognized in patients with HSP. Indeed, the prevalence of low urinary tract symptoms in HSP population was reported at 77.6% in a recent Estonian study. […] The study analyzed retrospectively the medical records of HSP patients admitted to the neurological rehabilitation unit of a tertiary care institute, from January 1999 to December 2009, and who had undergone a urodynamic investigation (UDI). […] All the patients voided spontaneously and none of them had any specific urological management before the UDI.

• #1 AP-4-Associated Hereditary Spastic Paraplegia (AP-4-HSP) | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/ap-4-associated-hereditary-spastic-paraplegia

  AP-4 hereditary spastic paraplegia (HSP), also known as AP-4 deficiency syndrome, is a group of slowly progressing neurodegenerative disorders. Children with these disorders may have developmental delays, moderate to severe intellectual disability, impaired or absent speech, small head size (microcephaly), seizures, and progressive motor symptoms. […] Most children born with AP-4-HSP have low muscle tone (hypotonia) in infancy that develops into high muscle tone (hypertonia) in early childhood. This causes spasticity of the legs that leads to the inability to walk and reliance on a wheelchair. Spasticity may also progress to the arms and hands, leading to the partial or total loss of use of all four limbs and torso (tetraplegia). […] There is no cure for AP-4-HSP. Treatment involves management of symptoms, which may include referrals to the following programs and services.

• #1 AP-4-Associated Hereditary Spastic Paraplegia (AP-4-HSP) | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/ap-4-associated-hereditary-spastic-paraplegia

  Children with AP-4-HSP should be referred to an early intervention (EI) program for access to occupational, physical, speech, and feeding therapy as well as mental health services, special educators, and sensory-impairment specialists. […] The child’s local public school district should develop an IEP based on the child’s level of function. The IEP will specify which instruction or related services the individual needs. […] At Boston Children’s Hospital, we care for patients with AP-4-HSP in the Department of Neurology, the Neuromuscular Center, the Cerebral Palsy Program, and the Clinical Genetics program. […] The Cerebral Palsy and Spasticity Center’s focus is to maximize children’s functional independence, stability, and quality of life. […] The Neuromuscular Center cares for infants, children, and teens with neuromuscular disorders. […] Pediatric Rehabilitation Medicine Program physicians focus on optimizing day-to-day function for children.

• #1 Management of Hereditary Spastic Paraplegia: A Systematic Review of the Literature

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6349696/

  The term hereditary spastic paraplegia (HSP) embraces a clinically and genetically heterogeneous group of neurodegenerative diseases characterized by progressive spasticity and weakness of the lower limbs. […] There currently exist no specific therapies for HSP, and treatment is exclusively symptomatic, aimed at reducing muscle spasticity, and improving strength and gait. […] Treatment is exclusively symptomatic and aimed mainly at reducing muscle spasticity and urinary urgency, and improving strength and gait. […] Therapeutic options include physical therapy, oral antispastic drugs (baclofen, progabide, dalfampridine), botulinum toxin therapy, and surgical baclofen pump implantation. […] Despite recent advances in understanding of the pathogenesis of HSP and the possibility, in several centers, of obtaining more precise and rapid molecular diagnoses, there is still no adequate evidence base for recommending the various published therapies.

• #1 Hereditary Spastic Paraplegia: Practice Essentials, Etiology, Epidemiology

  https://emedicine.medscape.com/article/306713-overview

  Currently, no specific treatment exists to prevent, retard, or reverse progressive disability in patients with HSP. Nonetheless, treatment approaches used for chronic paraplegia from other causes are useful. […] Regular physical therapy (PT) is important for maintaining and improving range of motion (ROM) and muscle strength. Furthermore, PT is necessary to maintain aerobic conditioning of the cardiovascular system. […] The types of exercise incorporated into PT programs for patients with HSP may include strengthening, stretching, and aerobic exercises.

• #1 Hereditary spastic paraplegia (HSP) for teenagers – Resource Library – Sheffield Children’s NHS Foundation Trust

  https://library.sheffieldchildrens.nhs.uk/hereditary-spastic-paraplegia-hsp-for-teenagers/

  You might come into contact with an occupational therapist. They can give advice and come up with practical solutions to help you with independent living, making activities easier to achieve. You can ask to be referred to an occupational therapist through your medical consultant or through other therapists such as a physiotherapist whom you might be seeing. […] Looking after your physical health is important to maintain your strength and abilities. The physiotherapist supports the multi-disciplinary team to review these and help to work with you and any other therapists involved in your care to support you. This will include: improving your functional ability, managing any tightness of your muscles, exercises. […] These exercises may involve stretching and strengthening of the lower limbs, cardiovascular (heart and lungs) as part of your daily routine and activities. […] HSP Group offer information and support helps improve the quality of life for those affected by HSP. Their friendly support community helps with sharing ideas and reducing feelings of isolation. They support research into HSP, including developing treatments and improving understanding.

• #1 Hereditary Spastic Paraplegia: What It Is, Symptoms & Types

  https://my.clevelandclinic.org/health/diseases/hereditary-spastic-paraplegia

  Hereditary spastic paraplegia is a genetic condition that causes weakness and muscle tightness in your legs. Treatment options, like medications and physical therapy, are available to help you manage symptoms as they arise. […] You may need mobility assistance, like with a cane, walker or wheelchair, years after you first notice symptoms. […] The activities you once could do are now difficult or unsafe. This can have a significant impact on your mental health. It may lead to stress and depression. Don’t hesitate to contact your healthcare provider if you notice you’re having more bad days than good ones. They may recommend you speak with a mental health professional to help you manage how this progressive condition affects your emotional well-being. […] There isn’t a cure available for hereditary spastic paraplegia, but treatment can help you manage symptoms, which may include: Medications (muscle relaxants, botulinum toxin injections, baclofen), Physical therapy, Occupational therapy, Medical shoe inserts (orthotics), Mobility devices, like a cane, crutches, splints or a wheelchair. […] Let a healthcare provider know if your symptoms worsen or if you notice any new symptoms. Your provider may adjust your treatment to help you manage this progressive condition.

• #1 Experienced complaints, activity limitations and loss of motor capacities in patients with pure hereditary spastic paraplegia: a web-based survey in the Netherlands | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-1338-4

  Hereditary spastic paraplegia (HSP) is a group of inherited disorders characterized by progressive spastic paresis of the lower limbs. Treatment is often focused on reducing spasticity and its physical consequences. To better address individual patients needs, we investigated a broad range of experienced complaints, activity limitations, and loss of motor capacities in pure HSP. […] Our results emphasize the large impact of spastic paraparesis on the lives of people with pure HSP and contribute to a better understanding of possible targets for rehabilitation. […] Thus, gaining more knowledge of SP-related complaints, activity limitations, and loss of motor capacities as experienced by patients is important for better disease management and tailoring interventions to individual patients needs.

• #1 Experienced complaints, activity limitations and loss of motor capacities in patients with pure hereditary spastic paraplegia: a web-based survey in the Netherlands | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-1338-4

  Unfortunately, the present data do not allow us to make inferences on the effect of walking aids on falls, but many participants reported the use of different types of walking aids to increase their walking distance. […] Based on the results of this study, it is not possible to conclude which walking aid and/or orthosis is generally most effective. […] Our results further showed that pain, fatigue, and autonomic problems are major (non-motor) symptoms in patients with pure HSP. […] Our results indicate that people with HSP form no exception to this rule, and probably remain undertreated in these respects. […] Hence, also for sexual problems, a combination of urological / gynecological consultation and adequate spasticity management seems to be crucial. […] The results of this web-based survey indicate that people with pure HSP experience many physical complaints, activity limitations, and loss of motor capacities. Of these, muscle stiffness, limited standing and walking activities, and increased fall risk are most prominent, but also pain, fatigue, autonomic dysfunctions, fear of falling, and problems with performing working and hobby activities are relevant symptoms and/or areas of disability with an inherent loss of quality of life.

• #1 Hereditary Spastic Paraparesis/Paraplegia – Child Neurology Foundation

  https://www.childneurologyfoundation.org/disorder/hereditary-spastic-paraplegia/

  Hereditary Spastic Paraparesis/Paraplegia (HSP) causes difficulty walking. In HSP, a person has tight and weak muscles. These tight muscles are also called spastic muscles. This is how the disorder gets its name. […] HSP is a genetic disorder. It can be caused by a mutation in any of a large number of genes. […] Symptoms of HSP can range from very mild to severe. The amount of muscle weakness a child experiences can vary. Some will have no weakness, or full strength. Others will have severe weakness, or paraplegia. […] The symptoms of HSP may worsen over time. Individuals with severe symptoms may lose the ability to walk independently. […] Treatment plans are guided by the symptoms of HSP. Some children may not require treatment. These children may be monitored by neurologist on a yearly basis depending on their symptoms.

• #1 Hereditary spastic paraplegia | MedLink Neurology

  https://www.medlink.com/handouts/hereditary-spastic-paraplegia

  Hereditary spastic paraplegia (HSP), also known as familial spastic paraparesis, refers to a group of inherited disorders that involves weakness and spasticity, which is stiffness of the legs. These symptoms get worse over time. […] Treatments such as muscle relaxers can sometimes relieve spasticity. Regular physical therapy is important for muscle strength and to keep range of motion. […] Consider participating in a clinical trial so clinicians and scientists can learn more about HSP and related disorders. Clinical research uses human volunteers to help researchers learn more about a disorder and perhaps find better ways to safely detect, treat, or prevent disease. […] The following organizations and resources can help individuals, families, friends, and caregivers of people living with HSP: Genetic Alliance, National Organization for Rare Disorders (NORD), Spastic Paraplegia Foundation.

• #1 Hereditary Spastic Paraplegia | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/hereditary-spastic-paraplegia

  Hereditary spastic paraplegia (HSP) refers to a group of over 80 different genetic conditions that present with progressive spasticity (muscle tightness) and weakness. Many forms of HSP are rare or even ultra-rare diseases. […] The primary symptom of HSP is difficulty walking due to spasticity and weakness in the legs. Both legs are affected, usually to a relatively similar degree. […] There is currently no cure for HSP. Available treatment can reduce symptoms and prevent complications. This may include physical therapy, occupational therapy, speech therapy, and other developmental supports; medications that reduce spasticity, injection of botulinum toxin, orthotics, and certain surgical procedures. […] Treatment requires an interdisciplinary approach, which may include referrals to several programs and services. The Movement Disorders Program at Boston Children’s Hospital is home to specialists with a variety of backgrounds and areas of focus. As a team, this diversity makes us better prepared to care for each child’s unique needs. […] At Boston Children’s Hospital, we care for patients with HSP in the Movement Disorders Program at the Department of Neurology, and in some case, the Neuromuscular Center, the Cerebral Palsy and Spasticity Center, and the Clinical Genetics Program.

• #1 Hereditary Spastic Paraplegia | Conditions | Liverpool Neuro Physio

  https://www.liverpoolneurophysio.co.uk/conditions-we-treat/hereditary-spastic-paraplegia.php

  Hereditary spastic paraplegia causes motor neurons in the brain and spinal cord to waste away or die, so they can no longer transmit impulses to the muscles. […] Your physiotherapist will provide you with the quality of care you need in order to address the functional difficulties that may arise as a result of HSP. […] In both types of HSP, physiotherapy can help manage muscle spasticity and weakness. […] At Liverpool Neuro your physiotherapist will provide you with an effective plan of physiotherapy treatment which will focus on improving and maintaining your strength in the muscles affected by HSP. […] The aim of your physiotherapist at Liverpool Neuro Physio is to address the functional difficulties that may arise as a result of hereditary spastic paraplegia in order to maximise your muscle function, functional ability and general health.

• #1 Desflurane for management of decompressive laminectomy in a patient with hereditary spastic paraplegia: a case report | JA Clinical Reports | Full Text

  https://jaclinicalreports.springeropen.com/articles/10.1186/s40981-019-0250-1

  Hereditary spastic paraplegia (HSP) is a rare, genetic neurodegenerative condition. Thus far, ideal anesthetic management is not established for patients with HSP; therefore, careful selection and dosage of anesthetic agents is required. […] Our successful experience in this case implies that, for patients with neuromuscular diseases, including HSP, desflurane may be an option for anesthetic management; moreover, careful assessment (e.g., medical condition, bispectral index, and train-of-four) should be performed prior to administration of anesthesia. […] HSP is associated with multiple difficulties in anesthetic management because it involves neurodegenerative disease; therefore, it is important to correctly establish the pathophysiology of affected patients. […] For safe anesthetic management, adequate assessment of the patients disease is needed, in order to correctly judge the risks and benefits of each anesthetic strategy.

• #1

  https://journals.lww.com/joac/fulltext/2022/12010/neuraxial_anesthesia_for_a_laboring_patient_with.12.aspx

  Hereditary spastic paraplegia (HSP) is a rare, inherited condition affecting the corticospinal tract, typically characterized by bilateral lower extremity weakness and stiffness. […] HSP presents unique anesthetic challenges, especially in the obstetric population. […] Here, we describe the successful use of an epidural technique for both labor analgesia and subsequent cesarean section. […] A multidisciplinary meeting took place involving the obstetric, anesthesiology, and neurology services. We decided to proceed with the induction of labor with the placement of an epidural catheter for labor analgesia. […] Neuraxial anesthesia obviates the need for muscle relaxants and airway concerns associated with general anesthesia. […] Epidural anesthesia appears to be a safe option for both labor analgesia and cesarean delivery in HSP patients. The utilization of neuraxial techniques avoids the risks associated with airway manipulation in a parturient as well as the need for muscle relaxants in the setting of an upper motor neuron disease.

• #1 Hereditary spastic paraplegia – Support for neurological conditions | The Brain Charity

  https://www.thebraincharity.org.uk/condition/hereditary-spastic-paraplegia/

  Hereditary spastic paraplegia is the name given to a group of rare disorders that are characterised by weakness and stiffness in the leg muscles. […] If you’re affected by hereditary spastic paraplegia, The Brain Charity can support you. […] We provide practical help on all aspects of living with hereditary spastic paraplegia, emotional support such as counselling, phone befriending and group therapy and social activities to people with hereditary spastic paraplegia from all over the UK from our centre in Liverpool. […] As there are limited treatment options, a great way to care for your loved one is helping with mobility, and being a companion. […] The Brain Charity provides free support for carers, friends and family of people with any form of neurological condition, including hereditary spastic paraplegia, from anywhere in the UK.

• #1 Management of Hereditary Spastic Paraplegia: A Systematic Review of the Literature

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6349696/

  Well-designed RCTs are needed to evaluate the efficacy of both symptomatic and pathogenetic treatments. […] The effectiveness of physical therapy in patients with HSP was found to be documented only in a small number of case reports and uncontrolled studies. […] There exist no clear indications on appropriate types and timing of physical therapy in HSP. […] We did not find high quality studies documenting the effectiveness of interventional and surgical therapies in patients with HSP. […] Overall, the quality of these studies was rated as low or very low, showing that there is still a lack of recommendations on the use of ITB in HSP. […] On the basis of the above studies, it can be concluded that SDR may possibly be a feasible option for alleviating spinal-related spasticity in uncomplicated HSP, while its effect in complicated HSP seems to be less predictable.

• #1 What Is Hereditary Spastic Paraplegia? An Overview

  https://drchandrilchugh.com/hereditary-spastic-paraplegia/what-is-hereditary-spastic-paraplegia-an-overview/

  Hereditary spastic paraplegia (HSP) has no cure, but several treatments can help. These treatments aim to make life better for people with this rare condition. They work to lower muscle stiffness, keep muscles strong, and support more independence. […] Physical therapy is another key part of managing HSP. Working with a physical therapist regularly keeps your muscles strong. It also helps you move better, which keeps you doing things on your own. […] Physical therapy for HSP often focuses on leg muscle strength and stretching. This helps prevent muscles from getting too tight. It also includes balance exercises to lower the chance of falling. Occupational therapy is also important. It teaches you how to adjust your home and daily activities so you can be more independent. […] Treating HSP needs a team. This includes doctors, therapists, and sometimes surgeons. They work together to come up with a plan just for you. With this team, living with HSP can be more manageable, and life can get better. […] Physical therapy is important for HSP patients. It helps keep muscles strong, improves movement, and maintains flexibility.

• #2 Spastic paraplegia type 7: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/spastic-paraplegia-type-7/

  Spastic paraplegia type 7 (also called SPG7) is one of more than 80 genetic disorders known as hereditary spastic paraplegias. These disorders primarily affect the brain and spinal cord (central nervous system), specifically nerve cells (neurons) that extend down the spinal cord. These neurons are used for muscle movement and sensation. Signs and symptoms of hereditary spastic paraplegias are characterized by progressive muscle stiffness (spasticity) in the legs and difficulty walking. […] Like all hereditary spastic paraplegias, spastic paraplegia type 7 involves spasticity of the leg muscles and some muscle weakness. People with this form of spastic paraplegia can also have ataxia; a pattern of movement abnormalities known as parkinsonism; exaggerated reflexes (hyperreflexia) in the arms; speech difficulties (dysarthria); difficulty swallowing (dysphagia); involuntary movements of the eyes (nystagmus); mild hearing loss; abnormal curvature of the spine (scoliosis); high-arched feet (pes cavus); numbness, tingling, or pain in the arms and legs (sensory neuropathy); disturbance in the nerves used for muscle movement (motor neuropathy); and muscle wasting (amyotrophy). The onset of symptoms varies greatly among those with spastic paraplegia type 7; however, abnormalities in muscle tone and other features usually become noticeable in adulthood.

• #2 NGS337: Spastic Paraplegia (NGS Panel and Copy Number Analysis + mtDNA) | MNG

  https://mnglabs.labcorp.com/tests/NGS337/spastic-paraplegia-ngs-panel-and-copy-number-analysis-mtdna

  The hereditary spastic paraplegias (HSPs) are clinically and genetically heterogeneous disorders characterized by lower extremity spasticity and weakness occurring in variable proportion. […] HSP is classified as „uncomplicated” if neurologic impairment is limited to lower extremity spastic weakness, hypertonic urinary bladder disturbance, and mild diminution of lower extremity vibration sensation. […] HSP is classified as „complicated” if the impairment present in uncomplicated HSP is accompanied by other systemic or neurologic abnormalities such as ataxia, seizures, cognitive impairment, dementia, amyotrophy, extrapyramidal disturbance, or peripheral neuropathy (in the absence of other causes for these additional features). […] Given the array of inheritance patterns (Autosomal recessive, X-linked, Autosomal dominant), a careful family history is important, particularly when interpreting genetic testing results.

• #2 Management of Hereditary Spastic Paraplegia: A Systematic Review of the Literature

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6349696/

  The term hereditary spastic paraplegia (HSP) embraces a clinically and genetically heterogeneous group of neurodegenerative diseases characterized by progressive spasticity and weakness of the lower limbs. […] There currently exist no specific therapies for HSP, and treatment is exclusively symptomatic, aimed at reducing muscle spasticity, and improving strength and gait. […] Treatment is exclusively symptomatic and aimed mainly at reducing muscle spasticity and urinary urgency, and improving strength and gait. […] Therapeutic options include physical therapy, oral antispastic drugs (baclofen, progabide, dalfampridine), botulinum toxin therapy, and surgical baclofen pump implantation. […] Despite recent advances in understanding of the pathogenesis of HSP and the possibility, in several centers, of obtaining more precise and rapid molecular diagnoses, there is still no adequate evidence base for recommending the various published therapies.

• #2 Paraplegia: Nursing Diagnoses & Care Plans | NurseTogether

  https://www.nursetogether.com/paraplegia-nursing-diagnosis-care-plan/

  Paraplegia is characterized by paralysis and loss of sensation in the lower extremities that occurs after a spinal cord injury. With paraplegia, autonomic dysreflexia can occur as an overreaction of the patients autonomic nervous system from noxious stimuli, resulting in potentially life-threatening hypertension. […] The management of paraplegia often focuses on preventing complications and further injuries. Immediate medical treatment can prevent partial paraplegia from becoming complete paraplegia. Physical and rehabilitation therapy is vital to strengthening muscles and alleviating pain. The nurse can support and encourage lifestyle modifications to empower patients to live actively and productively. […] Once the nurse identifies nursing diagnoses for paraplegia, nursing care plans help prioritize assessments and interventions for both short and long-term goals of care. […] Paraplegic patients have a higher risk for injuries because of the loss of function and sensation. […] Rehabilitation programs for paraplegic patients are available to provide support, maintain muscle strength and mass, and teach techniques to maneuver and perform tasks safely and independently.

• #2 Hereditary spastic paraplegia (HSP) | Paris Brain Institute

  https://parisbraininstitute.org/disease-files/hereditary-spastic-paraplegia-hsp

  Hereditary spastic paraplegia (HSP), also known as Strmpell-Lorrain disease, is a highly heterogeneous group of diseases. […] The main symptom of the disease is stiffness (spasticity) and weakness of the lower limbs, leading to severe walking difficulties due to damage to the motor neurons. […] These diseases are said to be chronic, progressing at different rates depending on the patient, with some patients becoming totally unable to walk and others showing very few symptoms several years after diagnosis. […] In the pure forms of the disease, which are the most common forms, symptoms include stiffness, spasticity and weakness of the lower limbs, leading to severe walking difficulties. […] Spastic paraplegia is diagnosed on the basis of a clinical examination, a study of the family history and a genetic analysis to find a causative mutation. […] There is currently no cure for these conditions, but treating symptoms such as spasticity can bring relief to sufferers. Physiotherapy, occupational therapy, balneotherapy and treatments based on muscle relaxants or botulinum toxin can help improve motor skills.

• #2 AP-4-Associated Hereditary Spastic Paraplegia (AP-4-HSP) | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/ap-4-associated-hereditary-spastic-paraplegia

  Children with AP-4-HSP should be referred to an early intervention (EI) program for access to occupational, physical, speech, and feeding therapy as well as mental health services, special educators, and sensory-impairment specialists. […] The child’s local public school district should develop an IEP based on the child’s level of function. The IEP will specify which instruction or related services the individual needs. […] At Boston Children’s Hospital, we care for patients with AP-4-HSP in the Department of Neurology, the Neuromuscular Center, the Cerebral Palsy Program, and the Clinical Genetics program. […] The Cerebral Palsy and Spasticity Center’s focus is to maximize children’s functional independence, stability, and quality of life. […] The Neuromuscular Center cares for infants, children, and teens with neuromuscular disorders. […] Pediatric Rehabilitation Medicine Program physicians focus on optimizing day-to-day function for children.

• #2 Hereditary spastic paraplegia | PPT

  https://www.slideshare.net/slideshow/hereditary-spastic-paraplegia/9334375

  Hereditary spastic paraplegia (HSP) is a group of inherited neurological disorders characterized by the progressive stiffening and weakening of the lower limbs. While there is no cure, treatment focuses on physical therapy, medication to reduce spasticity and pain, and addressing complications like contractures. […] Rehabilitation programme Regular physical therapy (PT) is important for maintaining and improving range of motion (ROM) and muscle strength. PT does not reduce the degenerative process within the spinal cord. The exercise programs include strengthening, stretching, and aerobic exercises. […] Medication The goals of pharmacotherapy are to reduce morbidity and prevent complications. Antispasmotic drugs Eg-Oxybutynin inhibits the action of acetylcholine on smooth muscle. […] This agent is most useful for treating spasticity in the gastrocnemius and soleus muscles.

• #2 Hereditary Spastic Paraplegia – Neuro Therapy For HSP

  https://www.physiofunction.co.uk/conditions/neurological/hereditary-spastic-paraplegia

  Although there isnt currently a cure for Hereditary Spastic Paraplegia, there are treatments and therapies to help manage the condition and maintain a good quality of life. […] PhysioFunctions highly experienced neurological physiotherapists, rehabilitation therapists, sports therapists and personal trainers are able to deliver tailored physiotherapy and exercise programs for clients who have HSP. […] Treatment of the condition is based around the management of the symptoms weakness and spasticity. […] Physiotherapy aims to optimise and improve cardiovascular fitness, optimise and improve muscle strength, improve mechanics for walking, balance, falls prevention and function, and maximise the individuals independence. […] Physiotherapy to maintain strength and mobility. […] Exercise Therapy to improve cardiovascular fitness and Soft tissue massage techniques and stretching to improve stiffness and spasticity.

• #2 Hereditary spastic paraplegia (HSP) for teenagers – Resource Library – Sheffield Children’s NHS Foundation Trust

  https://library.sheffieldchildrens.nhs.uk/hereditary-spastic-paraplegia-hsp-for-teenagers/

  You might come into contact with an occupational therapist. They can give advice and come up with practical solutions to help you with independent living, making activities easier to achieve. You can ask to be referred to an occupational therapist through your medical consultant or through other therapists such as a physiotherapist whom you might be seeing. […] Looking after your physical health is important to maintain your strength and abilities. The physiotherapist supports the multi-disciplinary team to review these and help to work with you and any other therapists involved in your care to support you. This will include: improving your functional ability, managing any tightness of your muscles, exercises. […] These exercises may involve stretching and strengthening of the lower limbs, cardiovascular (heart and lungs) as part of your daily routine and activities. […] HSP Group offer information and support helps improve the quality of life for those affected by HSP. Their friendly support community helps with sharing ideas and reducing feelings of isolation. They support research into HSP, including developing treatments and improving understanding.

• #2 Hereditary Spastic Paraplegia: What It Is, Symptoms & Types

  https://my.clevelandclinic.org/health/diseases/hereditary-spastic-paraplegia

  Hereditary spastic paraplegia is a genetic condition that causes weakness and muscle tightness in your legs. Treatment options, like medications and physical therapy, are available to help you manage symptoms as they arise. […] You may need mobility assistance, like with a cane, walker or wheelchair, years after you first notice symptoms. […] The activities you once could do are now difficult or unsafe. This can have a significant impact on your mental health. It may lead to stress and depression. Don’t hesitate to contact your healthcare provider if you notice you’re having more bad days than good ones. They may recommend you speak with a mental health professional to help you manage how this progressive condition affects your emotional well-being. […] There isn’t a cure available for hereditary spastic paraplegia, but treatment can help you manage symptoms, which may include: Medications (muscle relaxants, botulinum toxin injections, baclofen), Physical therapy, Occupational therapy, Medical shoe inserts (orthotics), Mobility devices, like a cane, crutches, splints or a wheelchair. […] Let a healthcare provider know if your symptoms worsen or if you notice any new symptoms. Your provider may adjust your treatment to help you manage this progressive condition.

• #2 Bladder dysfunction in hereditary spastic paraplegia: a clinical and urodynamic evaluation | Spinal Cord

  https://www.nature.com/articles/sc2011193

  UDI is essential in differentiating various urological disturbances that lead to the same clinical symptoms. […] HSP patients presenting a low urinary tract symptom should undergo a UDI to determine the exact micturition disturbances, initiate the appropriate treatments and avoid complications. […] Despite the presence of DO and DSD, HSP patients do not seem to have a high risk of developing ultrasonographically-assessed upper urinary tract complications after a mean follow-up of 22 years, contrary to spinal cord injury population. These results may guide practitioners in their decision-making about the appropriate evaluation and treatment of bladder disturbances that accompany hereditary spastic paraplegia.

• #2 Hereditary spastic paraplegia (HSP) for teenagers – Resource Library – Sheffield Children’s NHS Foundation Trust

  https://library.sheffieldchildrens.nhs.uk/hereditary-spastic-paraplegia-hsp-for-teenagers/

  Hereditary spastic paraplegia (HSP) is a general term for a group of rare inherited disorders that cause weakness and stiffness in the leg muscles. Estimates range from 1 in 11,000 people to 1 in 77,000 people. Symptoms will vary from person to person. […] Symptoms usually weakness in the legs and involuntary spasms and muscle stiffness (spasticity). There may be other symptoms in addition to the muscle weakness and spasticity, such as: a gradual weakness in the legs, increased muscle tone and stiffness (spasticity), problems peeing, such as an urgent need to pee, even when the bladder is not full, sometimes a lack of sensation in the feet. […] Children may develop leg stiffness and problems walking, such as stumbling and tripping, particularly on uneven ground. Some people may eventually need to use a walking aid or a wheelchair to help them get around. Others may not need to use any type of mobility equipment.

• #2 Hereditary Spastic Paraplegia | National Institute of Neurological Disorders and Stroke

  https://www.ninds.nih.gov/health-information/disorders/hereditary-spastic-paraplegia

  Currently, no treatments can prevent, slow, or reverse HSP. However, treatments are available to help manage symptoms. For example, muscle relaxers may help reduce stiffness in the legs. Some people benefit from surgery to loosen tight muscles. Assistive devices like braces, walkers, or wheelchairs can also improve mobility. Regular physical therapy can help people with HSP maintain muscle strength and flexibility. […] The outlook for people with HSP varies. Some people experience severe disability, while others have only mild symptoms. Most people with HSP have a normal life expectancy.

• #2 Experienced complaints, activity limitations and loss of motor capacities in patients with pure hereditary spastic paraplegia: a web-based survey in the Netherlands | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-1338-4

  Unfortunately, the present data do not allow us to make inferences on the effect of walking aids on falls, but many participants reported the use of different types of walking aids to increase their walking distance. […] Based on the results of this study, it is not possible to conclude which walking aid and/or orthosis is generally most effective. […] Our results further showed that pain, fatigue, and autonomic problems are major (non-motor) symptoms in patients with pure HSP. […] Our results indicate that people with HSP form no exception to this rule, and probably remain undertreated in these respects. […] Hence, also for sexual problems, a combination of urological / gynecological consultation and adequate spasticity management seems to be crucial. […] The results of this web-based survey indicate that people with pure HSP experience many physical complaints, activity limitations, and loss of motor capacities. Of these, muscle stiffness, limited standing and walking activities, and increased fall risk are most prominent, but also pain, fatigue, autonomic dysfunctions, fear of falling, and problems with performing working and hobby activities are relevant symptoms and/or areas of disability with an inherent loss of quality of life.

• #2 Hereditary Spastic Paraparesis – Advanced Neuro Rehab

  https://advancedneurorehab.com.au/condition/hereditary-spastic-paraparesis/

  Hereditary Spastic Paraparesis (HSP) is a group of inherited, degenerative neurological disorders that affect the upper motor neurons. […] Unfortunately there is no known medical treatment to slow or reverse HSP, however symptoms can be managed with medication and physiotherapy. […] Our physiotherapists at Advanced Neuro Rehab can help guide you through the management of your HSP. Education and advocacy are two important parts of the physiotherapists role. […] Physical management of HSP often involves maintaining independence and reducing any pain. […] Balance and walking can become difficult due to spasticity and movement compensations. Balance and gait re-education can allow you to improve your postural control and improve your walking.

• #2 Hereditary spastic paraplegia Treatments | Expert Care

  https://ksnr.org.in/hereditary-spastic-paraparesis/

  Hereditary Spastic Paraplegia: Comprehensive Care and Support […] Our specialized HSP clinic offers a range of innovative therapy options designed to manage symptoms and improve quality of life: […] Customized physical therapy programs to maintain muscle strength and flexibility […] Comprehensive support services include: […] Expert counseling to help you cope with the emotional aspects of HSP […] Support groups connecting you with others who understand your journey […] Educational resources to keep you informed about the latest HSP research and treatments.

• #2 Hereditary spastic paraplegia | MedLink Neurology

  https://www.medlink.com/handouts/hereditary-spastic-paraplegia

  Hereditary spastic paraplegia (HSP), also known as familial spastic paraparesis, refers to a group of inherited disorders that involves weakness and spasticity, which is stiffness of the legs. These symptoms get worse over time. […] Treatments such as muscle relaxers can sometimes relieve spasticity. Regular physical therapy is important for muscle strength and to keep range of motion. […] Consider participating in a clinical trial so clinicians and scientists can learn more about HSP and related disorders. Clinical research uses human volunteers to help researchers learn more about a disorder and perhaps find better ways to safely detect, treat, or prevent disease. […] The following organizations and resources can help individuals, families, friends, and caregivers of people living with HSP: Genetic Alliance, National Organization for Rare Disorders (NORD), Spastic Paraplegia Foundation.

• #2 Diagnosed – Hereditary Spastic Paraplegia – Primary progressive MS – MS Society UK | Forum

  https://forum.mssociety.org.uk/t/diagnosed-hereditary-spastic-paraplegia/23773

  I am the president of The Spastic Paraplegia Foundation, sp-foundation.org. Our mission is to find a cure for Hereditary Spastic Paraplegia. […] Dedicated to finding a cure for Hereditary Spastic Paraplegia or Strumpell-Lorrain Disease and Primary Lateral Sclerosis. We also offer resources, education and support for people with these diseases.

• #2 Hereditary Spastic Paraplegia | Conditions | Liverpool Neuro Physio

  https://www.liverpoolneurophysio.co.uk/conditions-we-treat/hereditary-spastic-paraplegia.php

  Hereditary spastic paraplegia causes motor neurons in the brain and spinal cord to waste away or die, so they can no longer transmit impulses to the muscles. […] Your physiotherapist will provide you with the quality of care you need in order to address the functional difficulties that may arise as a result of HSP. […] In both types of HSP, physiotherapy can help manage muscle spasticity and weakness. […] At Liverpool Neuro your physiotherapist will provide you with an effective plan of physiotherapy treatment which will focus on improving and maintaining your strength in the muscles affected by HSP. […] The aim of your physiotherapist at Liverpool Neuro Physio is to address the functional difficulties that may arise as a result of hereditary spastic paraplegia in order to maximise your muscle function, functional ability and general health.

• #2 Hereditary Spastic Paraplegia | Conditions | Liverpool Neuro Physio

  https://www.liverpoolneurophysio.co.uk/conditions-we-treat/hereditary-spastic-paraplegia.php

  Unfortunately it is impossible to prevent or slow down the degenerative process affecting the motor neurons within the spinal cord. However effective physiotherapy treatment allows individuals with hereditary spastic paraplegia to maintain their active range of movement for as long as possible which simultaneously maximises the individuals functional independence and quality of life. […] At Liverpool Neuro Physio your physiotherapist can provide you with an effective plan of treatment in order to help maintain your independent mobility and your functional independence and slow the progression of muscle weakness and wastage.

• #2 On-Pump Coronary Artery Bypass Grafting and Postoperative Cardiac ICU Care in a Patient with Hereditary Spastic Paraplegia: A Case Presentation – MedCrave online

  https://medcraveonline.com/JACCOA/on-pump-coronary-artery-bypass-grafting-and-postoperative-cardiac-icu-care-in-a-patient-with-hereditary-spastic-paraplegia-a-case-presentation.html

  Hereditary Spastic Paraplegia (HSP) is a rare condition with very little information in the literature regarding its implications for major surgery and perioperative management in critical care. […] We have been able to demonstrate that this units standard anaesthesia and postoperative care was safe and effective for this patient with HSP. […] HSP is an uncommon disease and there was no experience within our unit nor in the literature to guide our practice. […] We would recommend that general anaesthesia, including the use of opioids and NDMRs, is safe for on-pump cardiac surgery in patients with HSP. The same could also be said for similarly major forms of surgery requiring postoperative critical care management, with the caveat that the usual care and attention is paid to the reversal of NDMRs at the end of surgery where necessary.

• #2 Desflurane for management of decompressive laminectomy in a patient with hereditary spastic paraplegia: a case report | JA Clinical Reports | Full Text

  https://jaclinicalreports.springeropen.com/articles/10.1186/s40981-019-0250-1

  We suggest that desflurane, in combination with other routinely used and established anesthetics, is a satisfactory anesthetic agent for such patients. Importantly, desflurane may be an option for safe and effective anesthetic management if the patients disease condition and anesthetic states are closely and carefully monitored.

• #2

  https://journals.lww.com/joac/fulltext/2022/12010/neuraxial_anesthesia_for_a_laboring_patient_with.12.aspx

  Hereditary spastic paraplegia (HSP) is a rare, inherited condition affecting the corticospinal tract, typically characterized by bilateral lower extremity weakness and stiffness. […] HSP presents unique anesthetic challenges, especially in the obstetric population. […] Here, we describe the successful use of an epidural technique for both labor analgesia and subsequent cesarean section. […] A multidisciplinary meeting took place involving the obstetric, anesthesiology, and neurology services. We decided to proceed with the induction of labor with the placement of an epidural catheter for labor analgesia. […] Neuraxial anesthesia obviates the need for muscle relaxants and airway concerns associated with general anesthesia. […] Epidural anesthesia appears to be a safe option for both labor analgesia and cesarean delivery in HSP patients. The utilization of neuraxial techniques avoids the risks associated with airway manipulation in a parturient as well as the need for muscle relaxants in the setting of an upper motor neuron disease.

• #2 Hereditary spastic paraplegia – Support for neurological conditions | The Brain Charity

  https://www.thebraincharity.org.uk/condition/hereditary-spastic-paraplegia/

  All The Brain Charity’s services are free for carers and family members of people with neurological conditions too. […] We can help you apply for benefits like PIP and Universal Credit. […] The European Reference Network has developed a Patient Journey for Hereditary Spastic Paraplegias (HSPs). […] The Brain Charity is not aware of a support group currently running for this condition.

• #2 Management of Hereditary Spastic Paraplegia: A Systematic Review of the Literature

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6349696/

  Well-designed RCTs are needed to evaluate the efficacy of both symptomatic and pathogenetic treatments. […] The effectiveness of physical therapy in patients with HSP was found to be documented only in a small number of case reports and uncontrolled studies. […] There exist no clear indications on appropriate types and timing of physical therapy in HSP. […] We did not find high quality studies documenting the effectiveness of interventional and surgical therapies in patients with HSP. […] Overall, the quality of these studies was rated as low or very low, showing that there is still a lack of recommendations on the use of ITB in HSP. […] On the basis of the above studies, it can be concluded that SDR may possibly be a feasible option for alleviating spinal-related spasticity in uncomplicated HSP, while its effect in complicated HSP seems to be less predictable.

• #2 What Is Hereditary Spastic Paraplegia? An Overview

  https://drchandrilchugh.com/hereditary-spastic-paraplegia/what-is-hereditary-spastic-paraplegia-an-overview/

  Hereditary spastic paraplegia (HSP) has no cure, but several treatments can help. These treatments aim to make life better for people with this rare condition. They work to lower muscle stiffness, keep muscles strong, and support more independence. […] Physical therapy is another key part of managing HSP. Working with a physical therapist regularly keeps your muscles strong. It also helps you move better, which keeps you doing things on your own. […] Physical therapy for HSP often focuses on leg muscle strength and stretching. This helps prevent muscles from getting too tight. It also includes balance exercises to lower the chance of falling. Occupational therapy is also important. It teaches you how to adjust your home and daily activities so you can be more independent. […] Treating HSP needs a team. This includes doctors, therapists, and sometimes surgeons. They work together to come up with a plan just for you. With this team, living with HSP can be more manageable, and life can get better. […] Physical therapy is important for HSP patients. It helps keep muscles strong, improves movement, and maintains flexibility.

• #2 Best Hereditary Spastic Paraplegia Treatment near Bollaram

  https://www.medicoverhospitals.in/articles/best-hereditary-spastic-paraplegia-treatment-in-bollaram

  Living with hereditary spastic paraplegia can be challenging, impacting mobility and daily activities. Finding the right treatment is crucial for managing symptoms and improving quality of life. Near Bollaram, Medicover Hospitals offer advanced care options for individuals with this condition. Our focus is on pain management, symptom control, and safe recovery in a supportive environment. […] At Medicover Hospitals near Bollaram, our specialists conduct thorough evaluations to differentiate Hereditary Spastic Paraplegia from similar conditions. Accurate diagnosis is crucial for determining the best treatment plan. […] Medications are prescribed to manage symptoms such as muscle stiffness and spasticity. These medications help improve mobility and quality of life for individuals with Hereditary Spastic Paraplegia.

• #3 Best Hereditary Spastic Paraplegia Treatment near Bollaram

  https://www.medicoverhospitals.in/articles/best-hereditary-spastic-paraplegia-treatment-in-bollaram

  Therapies such as physical therapy and occupational therapy are essential in managing Hereditary Spastic Paraplegia. These therapies aim to improve strength, flexibility, and independence in daily activities. […] Advanced procedures like selective dorsal rhizotomy may be recommended in severe cases of Hereditary Spastic Paraplegia. These procedures target specific nerves to reduce muscle spasticity. […] At Medicover Hospitals near Bollaram, our specialists offer a comprehensive approach to Hereditary Spastic Paraplegia treatment. From medications to advanced procedures, we strive to provide the best quality care for our patients. […] Trust our specialists at Medicover Hospital for your well-being and safe recovery.

• #3 Hereditary Spastic Paraparesis – Advanced Neuro Rehab

  https://advancedneurorehab.com.au/condition/hereditary-spastic-paraparesis/

  Hereditary Spastic Paraparesis (HSP) is a group of inherited, degenerative neurological disorders that affect the upper motor neurons. […] Unfortunately there is no known medical treatment to slow or reverse HSP, however symptoms can be managed with medication and physiotherapy. […] Our physiotherapists at Advanced Neuro Rehab can help guide you through the management of your HSP. Education and advocacy are two important parts of the physiotherapists role. […] Physical management of HSP often involves maintaining independence and reducing any pain. […] Balance and walking can become difficult due to spasticity and movement compensations. Balance and gait re-education can allow you to improve your postural control and improve your walking.

• #3 What Is Hereditary Spastic Paraplegia? An Overview

  https://drchandrilchugh.com/hereditary-spastic-paraplegia/what-is-hereditary-spastic-paraplegia-an-overview/

  Hereditary spastic paraplegia (HSP) has no cure, but several treatments can help. These treatments aim to make life better for people with this rare condition. They work to lower muscle stiffness, keep muscles strong, and support more independence. […] Physical therapy is another key part of managing HSP. Working with a physical therapist regularly keeps your muscles strong. It also helps you move better, which keeps you doing things on your own. […] Physical therapy for HSP often focuses on leg muscle strength and stretching. This helps prevent muscles from getting too tight. It also includes balance exercises to lower the chance of falling. Occupational therapy is also important. It teaches you how to adjust your home and daily activities so you can be more independent. […] Treating HSP needs a team. This includes doctors, therapists, and sometimes surgeons. They work together to come up with a plan just for you. With this team, living with HSP can be more manageable, and life can get better. […] Physical therapy is important for HSP patients. It helps keep muscles strong, improves movement, and maintains flexibility.

• #3 Hereditary spastic paraplegia – Support for neurological conditions | The Brain Charity

  https://www.thebraincharity.org.uk/condition/hereditary-spastic-paraplegia/

  Hereditary spastic paraplegia is the name given to a group of rare disorders that are characterised by weakness and stiffness in the leg muscles. […] If you’re affected by hereditary spastic paraplegia, The Brain Charity can support you. […] We provide practical help on all aspects of living with hereditary spastic paraplegia, emotional support such as counselling, phone befriending and group therapy and social activities to people with hereditary spastic paraplegia from all over the UK from our centre in Liverpool. […] As there are limited treatment options, a great way to care for your loved one is helping with mobility, and being a companion. […] The Brain Charity provides free support for carers, friends and family of people with any form of neurological condition, including hereditary spastic paraplegia, from anywhere in the UK.

• #3

  https://journals.lww.com/joac/fulltext/2022/12010/neuraxial_anesthesia_for_a_laboring_patient_with.12.aspx

  Hereditary spastic paraplegia (HSP) is a rare, inherited condition affecting the corticospinal tract, typically characterized by bilateral lower extremity weakness and stiffness. […] HSP presents unique anesthetic challenges, especially in the obstetric population. […] Here, we describe the successful use of an epidural technique for both labor analgesia and subsequent cesarean section. […] A multidisciplinary meeting took place involving the obstetric, anesthesiology, and neurology services. We decided to proceed with the induction of labor with the placement of an epidural catheter for labor analgesia. […] Neuraxial anesthesia obviates the need for muscle relaxants and airway concerns associated with general anesthesia. […] Epidural anesthesia appears to be a safe option for both labor analgesia and cesarean delivery in HSP patients. The utilization of neuraxial techniques avoids the risks associated with airway manipulation in a parturient as well as the need for muscle relaxants in the setting of an upper motor neuron disease.

• #3 On-Pump Coronary Artery Bypass Grafting and Postoperative Cardiac ICU Care in a Patient with Hereditary Spastic Paraplegia: A Case Presentation – MedCrave online

  https://medcraveonline.com/JACCOA/on-pump-coronary-artery-bypass-grafting-and-postoperative-cardiac-icu-care-in-a-patient-with-hereditary-spastic-paraplegia-a-case-presentation.html

  Hereditary Spastic Paraplegia (HSP) is a rare condition with very little information in the literature regarding its implications for major surgery and perioperative management in critical care. […] We have been able to demonstrate that this units standard anaesthesia and postoperative care was safe and effective for this patient with HSP. […] HSP is an uncommon disease and there was no experience within our unit nor in the literature to guide our practice. […] We would recommend that general anaesthesia, including the use of opioids and NDMRs, is safe for on-pump cardiac surgery in patients with HSP. The same could also be said for similarly major forms of surgery requiring postoperative critical care management, with the caveat that the usual care and attention is paid to the reversal of NDMRs at the end of surgery where necessary.

• #3 Hereditary Spastic Paraplegia – Neuro Therapy For HSP

  https://www.physiofunction.co.uk/conditions/neurological/hereditary-spastic-paraplegia

  Although there isnt currently a cure for Hereditary Spastic Paraplegia, there are treatments and therapies to help manage the condition and maintain a good quality of life. […] PhysioFunctions highly experienced neurological physiotherapists, rehabilitation therapists, sports therapists and personal trainers are able to deliver tailored physiotherapy and exercise programs for clients who have HSP. […] Treatment of the condition is based around the management of the symptoms weakness and spasticity. […] Physiotherapy aims to optimise and improve cardiovascular fitness, optimise and improve muscle strength, improve mechanics for walking, balance, falls prevention and function, and maximise the individuals independence. […] Physiotherapy to maintain strength and mobility. […] Exercise Therapy to improve cardiovascular fitness and Soft tissue massage techniques and stretching to improve stiffness and spasticity.

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