Issn 23211822 case report physical therapy interventions for the patients with hereditary spastic paraparesisan exploratory case reports asir john samuel1, vencita priyanka aranha2, trapthi kamath3, monika pokhrel4. Pdf human tlymphotropic virus 1 htlv1associated myelopathytropical spastic paraparesis hamtsp is a progressive disease of the cns that causes. Hereditary spastic paraplegia hsp refers to a heterogeneous group of neurodegenerative conditions characterized by progressive degeneration of the corticospinal tracts and posterior column of the spinal cord. Tropical spastic paraparesis is listed as a rare disease by the office of rare diseases ord of the national institutes of health nih.
Hereditary spastic paraplegia is a general term for a group of rare inherited disorders that cause weakness and stiffness in the leg muscles. Tropical spastic paraparesis information page national. Its caused by the human tcell lymphotrophic virus type. We describe serial mr imaging findings in a patient with htlviassociated myelopathy. The two syndromes were determined to be the same disease and were termed. Although poorly recognised by neurologists, spastic paraparesis can be one of the results of some metabolism disorders in children and adults. Case report physical therapy interventions for the. Spastic paraplegia type 4 is a pure hereditary spastic paraplegia. Tropical spastic paraparesis in kerala, south india oomman a. Tropical spastic paraparesis an overview sciencedirect topics. Hereditary spastic paraparesis affects both sexes and may begin at any age.
Hereditary spastic paraparesis hsp or the strumpelllorrain syndrome is the name given to a heterogeneous group of inherited disorders in which the main clinical feature is progressive lower. Current neurology and neuroscience reports, 2006, 61. A case study r narismulu greys hospital february 2008. Bruhn, in mandell, douglas, and bennetts principles and practice of infectious diseases eighth edition, 2015.
Hereditary spastic paraplegia genetic and rare diseases. Tropical spastic paraparesis tsp, also known as htlv1associated myelopathy ham was described in jamaica as far back as 1897. Proposal for diagnostic criteria of tropical spastic paraparesishtlvi associated myelopathy tspham. As the name suggests, it is most common in tropical regions, including the caribbean. Spastic paraplegia type 3a is one of a group of genetic disorders known as hereditary spastic paraplegias. Blood transfusion products are screened for htlv1 antibodies, as a preventive measure. Hereditary spastic paraplegia spg is a group of neurodegenerative disorders characterized by progressive spasticity and weakness of the lower limbs, resulting from dying back axonopathy of the corticospinal tract. Pdf on jan 1, 1991, a g kermode and others published mri of thoracic cord in tropical spastic paraparesis find, read and cite all the research you need on researchgate. Gait instability, sensory deficits, weakness, spasticity, and back pain are main findings. It is usually caused by spinal cord injury or a congenital condition that affects the neural brain elements of the spinal canal. Pdf htlv1associated myelopathytropical spastic paraparesis. Treatment is symptomatic, including drugs to relieve spasticity. This means that tropical spastic paraparesis, or a subtype of tropical spastic paraparesis, affects less than 200,000 people in the us population.
In 1993, htlvii was isolated in the absence of any other detectable human retroviruses from a. Hereditary spastic paraplegias hsp are a rare group of inherited neurodegenerative diseases which mainly affect the longest axons of the corticospinal tract and the dorsal column which supply the lower limbs. Tropical spastic paraparesis is a still incompletely understood neurologic disorder caused by human tcell lymphotropic virus 1 htlv1, with a mean onset of symptoms in the fourth and fifth decade of life. Alzheimer disease, familial, 3, with spastic paraparesis and unusual plaques.
Human tlymphotropic virus type 1associated myelopathytropical. The chart showing pdf series, word series, html series, scan qr codes. Its infection occurs either vertically breastfeeding or horizontally through sexual intercourse, blood transfusion. Hsp is also known as hereditary spastic paraparesis, familial spastic paraplegia, french settlement disease, strumpell disease, or strumpelllorrain disease.
Idiopathic progressive spastic paraparesis jama jama. Paraplegia is an impairment in motor or sensory function of the lower extremities. To be updated when it is complete please like us on facebook, follow us on twitter or subscribe on youtube using the follow us buttons. Spastic paraplegia type 4 genetics home reference nih. Tropical spastic paraparesishtlv1associated myelopathy tsp. Htlv1 infection in afrocaribbean patients in the united kingdom. Hereditary spastic paraplegia hsp is a group of hereditary, degenerative, neurological disorders that primarily affect the upper motor neurons. Tropical spastic paraparesis, human tcell lymphotropic virus. Conditions listing spastic paraparesis as a symptom may also be potential underlying causes of spastic paraparesis. All forms cause degeneration of the nerve pathways that carry signals from the brain down the spinal cord to muscles. Paraparesis is a medical condition that is characterized by the weakness or paralysis partial of the lower extremities. Tropical spastic paraparesis tsp is a chronic degenerative neurological disease associated with this infection.
For several decades the term tropical spastic paraparesis tsp has been used to describe a chronic and progressive disease of the nervous system that affects adults living in equatorial areas of the world and causes progressive weakness, stiff muscles, muscle spasms, sensory disturbance, and sphincter dysfunction. Tropical spastic paraparesis tsp, a myeloneuropathy, was reported in the last few decades. If the patient can walk, look for a spastic gait with bilateral circumduction described as scissoring, and whilst they are standing inspect the spine for scars. Tropical spastic paraparesis definition tropical spastic paraparesis tsp is an incurable viral infection of the spinal cord that causes weakness in the legs. Webb s, coleman d, byrne p, parfrey n, burke t, hutchinson j, hutchinson m. Description as the name implies, tropical spastic paraparesis usually occurs in tropical locales. Tropical spastic paraparesis, human tlymphotropic virus 1, qualitative research, psychosocial approach introduction human tcell lymphotropic virus type 1 htlv1 was the first retrovirus identified in humans 1. There are two types of tropical myeloneuropathies that are different in aetiology and clinical features. Differential diagnosis for spastic paraparesis oxford. The patient had acute progression of neurologic symptoms and exhibited swelling of the entire length of the spinal cord with increased t2 signal and contrast enhancement on mr imaging. This type of paraparesis is known as familial spastic paraparesis, as develops from an inherited gene.
Multiple sclerosis, tropical spastic paraparesis and journal of. The area of the spinal canal that is affected in paraplegia is either the thoracic, lumbar, or sacral regions. Tropical spastic paraparesis tsp and htlviassociated myelopathy ham have also been associated with the dual infection of htlvi and hiv2 3, with hiv2 only 4, and with the combination of htlvi and htlvii 5. During the 80s, the htlvi associated myelopathytropical spastic paraparesis hamtsp was identified in regions such as the caribe, south africa, southern india, seychelles islands, ivory coast and southern japan. It is caused by the human tcell lymphotropic virus1 htlv1 retrovirus. Hereditary spastic paraplegia hsp, also called familial spastic paraparesis fsp, refers to a group of inherited disorders that are characterized by progressive weakness and spasticity stiffness of the legs. Metabolic cause of some spastic paraplegia hsp research. Proposal for diagnostic criteria of tropical spastic paraparesishtlviassociated myelopathy tspham. Like all hereditary spastic paraplegias, spastic paraplegia type 4 involves spasticity of the leg muscles and muscle weakness. Summary in order to evaluate if there is variation on the intensity of cerebral spinal fluid csf response during htlvi associated tropical spastic paraparesis. Hereditary spastic paraparesis brain, spinal cord, and. Tropical spastic paraparesis definition of tropical. Our database lists the following as having spastic paraparesis as a symptom of that condition. Please include your name and address, but these will be omitted on request.
Tropical spastic paraparesis tsp tsp is a disease of the nervous system that causes weakness, stiffness, and muscle spasms of the legs. Spastic paraplegia foundation spf 1605 goularte place fremont, ca 945397241 telephone. In a large italian pedigree with a new form of rare, autosomal dominant spastic paraparesis, seemingly identical to the disorder in the family reported by slavotinek et al. Hereditary spastic paraparesis hsp is a heterogeneous group of inherited disorders, with prevalence ranged from 4. They pointed out that several neurologic disorders map to the same region. The disease presents with progressive stiffness and contraction in the lower limbs. Look around the bed for walking aids or wheelchairs, and check for the presence of a catheter. Pdf proposal for diagnostic criteria of tropical spastic. Upper motor neurons in the brain and spinal cord deliver signals to the lower motor neurons, which in turn, carry messages to the muscles. Hereditary spastic paraplegia comprises a wide and heterogeneous group of inherited neurodegenerative and neurodevelopmental disorders resulting from primary retrograde dysfunction of the long descending fibers of the corticospinal tract. This disorder has many forms and can result from many different types of genetic abnormalities. People with this condition can also experience exaggerated reflexes hyperreflexia, ankle spasms, higharched feet pes cavus, and reduced bladder. Diagnosis is clinical and sometimes by genetic testing. Hereditary spastic paraplegia information page national.
Tropical spastic paraparesis zchronic viral myelitis can be associated. The hsp incidence rate in the united states is 20,000 people. They both occur predominantly in tropical countries, although tropical spastic paraparesis tsp has been described in temperate southern japan. Hsp is caused by defects in genes which code for proteins essential for fast axonal neurotransmission. The coexistence of multiple sclerosis and hereditary. Inspect wasting, fasciculations, wheelchairwalking aid, catheter, pes cavus gait and rhombergs test tone power reflexes sensation sensory level, joint position sense and vibration coordination if time allows and you suspect spastic paraparesis proceed as follows. Ham, or tropical spastic paraparesis, is a chronic progressive demyelinating disease that affects the spinal cord and white matter of the cns. Although spastic paraparesis and urinary dysfunction represent the most common clinical presentation, a complex group of different. Cervical myelopathy c5t1 anterior horn cell disease motor neuron disease syringomyelia. Adultonset, chronic progressive spastic paraparesis may be due to a large number of causes and poses a diagnostic challenge. Spastic paraplegia type 3a genetics home reference nih.
These disorders are characterized by muscle stiffness spasticity and weakness in the lower limbs paraplegia. Common and important causes of spastic paraparesis for doctors and medical students this page is currently being written and will be available soon. Hereditary spastic paraparesis neurologic disorders. Signs, meanings and practices of people living with human. Hereditary spastic paraparesis is a group of rare hereditary disorders characterized by progressive, spinal, nonsegmental spastic leg paresis, sometimes with intellectual disability, seizures, and other extraspinal deficits. Lecturer, alvas college of physiotherapy, moodabidri, india1. Familial spastic paraparesis, optic atrophy, and dementia. Those areas were found to be endemic for the htlvi, the etiologic agent for hamtsp. Autosomal dominant hereditary spastic paraparesis with cognitive loss linked to chromosome 2p. There are two forms of paraparesis which further explain the primary causes of the disease. Htlvi associated myelopathytropical spastic paraparesis ham.
Rare cases of tropical spastic paraparesis are caused by htlv2. The diagnosis is mainly based on history, clinical findings, and the detection of specific. Early in the disease course, there may be mild gait difficulties and stiffness. Htlv1 proviral load in infective dermatitis associated with. Htlv1 associated myelopathy tropical spastic paraparesis hamtsp is a progressive disease of the nervous system that affects less than 2% of people with htlv1 infection signs and symptoms vary but may include slowly progressive weakness and spasticity of one or both legs, exaggerated reflexes, muscle contractions in the ankle, and lower back pain. Hereditary spastic paraplegia hsp is a group of rare, inherited neurological disorders. Multiple sclerosis, tropical spastic paraparesis and.
Hereditary spastic paraplegia knowledge for medical. Possible etiologies for tropical spastic paraparesis and. If you have problems viewing pdf files, download the latest version of adobe reader. Pdf mri of thoracic cord in tropical spastic paraparesis.
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