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. Cervical myelopathy c5t1 anterior horn cell disease motor neuron disease syringomyelia. This type of paraparesis is known as familial spastic paraparesis, as develops from an inherited gene. Look around the bed for walking aids or wheelchairs, and check for the presence of a catheter. 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. Our database lists the following as having spastic paraparesis as a symptom of that condition.
Description as the name implies, tropical spastic paraparesis usually occurs in tropical locales. Familial spastic paraparesis, optic atrophy, and dementia. Paraparesis is a medical condition that is characterized by the weakness or paralysis partial of the lower extremities. Webb s, coleman d, byrne p, parfrey n, burke t, hutchinson j, hutchinson m. The area of the spinal canal that is affected in paraplegia is either the thoracic, lumbar, or sacral regions. This means that tropical spastic paraparesis, or a subtype of tropical spastic paraparesis, affects less than 200,000 people in the us population. Its infection occurs either vertically breastfeeding or horizontally through sexual intercourse, blood transfusion. Signs, meanings and practices of people living with human. We thank dr vitoria regina almeida rego, who provided us with. Paraparesis symptoms, causes, treatment, types, diagnosis. The coexistence of multiple sclerosis and hereditary. 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. Gait instability, sensory deficits, weakness, spasticity, and back pain are main findings. If you have problems viewing pdf files, download the latest version of adobe reader.
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. Metabolic cause of some spastic paraplegia hsp research. Pdf proposal for diagnostic criteria of tropical spastic. Tropical spastic paraparesishtlv1associated myelopathy tsp. Spastic paraplegia foundation spf 1605 goularte place fremont, ca 945397241 telephone.
Human tlymphotropic virus type 1associated myelopathytropical. Ham, or tropical spastic paraparesis, is a chronic progressive demyelinating disease that affects the spinal cord and white matter of the cns. Pdf htlv1associated myelopathytropical spastic paraparesis. Tropical spastic paraparesis tsp, also known as htlv1associated myelopathy ham was described in jamaica as far back as 1897. Spastic paraplegia type 4 genetics home reference nih. Hereditary spastic paraplegia hsp is a group of inherited diseases whose main feature is a progressive gait disorder. 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.
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. Multiple sclerosis, tropical spastic paraparesis and. Like all hereditary spastic paraplegias, spastic paraplegia type 4 involves spasticity of the leg muscles and muscle weakness. The two syndromes were determined to be the same disease and were termed. As the name suggests, it is most common in tropical regions, including the caribbean.
The disease presents with progressive stiffness and contraction in the lower limbs. 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. Although spastic paraparesis and urinary dysfunction represent the most common clinical presentation, a complex group of different. Pdf human tlymphotropic virus 1 htlv1associated myelopathytropical spastic paraparesis hamtsp is a progressive disease of the cns that causes. Alzheimer disease, familial, 3, with spastic paraparesis and unusual plaques. Htlv1 associated myelopathytropical spastic paraparesis. Tropical spastic paraparesis an overview sciencedirect topics. 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. Diagnosis is clinical and sometimes by genetic testing. Hereditary spastic paraparesis neurologic disorders.
The diagnosis is mainly based on history, clinical findings, and the detection of specific. Case report physical therapy interventions for the. Differential diagnosis for spastic paraparesis oxford. Tropical spastic paraparesis tsp, a myeloneuropathy, was reported in the last few decades. The hsp incidence rate in the united states is 20,000 people. Htlvi associated myelopathytropical spastic paraparesis ham. 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.
Tropical spastic paraparesis tsp tsp is a disease of the nervous system that causes weakness, stiffness, and muscle spasms of the legs. The chart showing pdf series, word series, html series, scan qr codes. Conditions listing spastic paraparesis as a symptom may also be potential underlying causes of spastic paraparesis. They pointed out that several neurologic disorders map to the same region. Although poorly recognised by neurologists, spastic paraparesis can be one of the results of some metabolism disorders in children and adults.
Tropical spastic paraparesis zchronic viral myelitis can be associated. Tropical spastic paraparesis information page national. There are two types of tropical myeloneuropathies that are different in aetiology and clinical features. We describe serial mr imaging findings in a patient with htlviassociated myelopathy. Blood transfusion products are screened for htlv1 antibodies, as a preventive measure. Spastic paraplegia type 3a genetics home reference nih. They both occur predominantly in tropical countries, although tropical spastic paraparesis tsp has been described in temperate southern japan. Pdf mri of thoracic cord in tropical spastic paraparesis. Autosomal dominant hereditary spastic paraparesis with cognitive loss linked to chromosome 2p.
These disorders are characterized by muscle stiffness spasticity and weakness in the lower limbs paraplegia. Spastic paraplegia type 3a is one of a group of genetic disorders known as hereditary spastic paraplegias. Proposal for diagnostic criteria of tropical spastic paraparesishtlviassociated myelopathy tspham. Hsp is also known as hereditary spastic paraparesis, familial spastic paraplegia, french settlement disease, strumpell disease, or strumpelllorrain disease. Htlv1 infection in afrocaribbean patients in the united kingdom. This disorder has many forms and can result from many different types of genetic abnormalities.
Rare cases of tropical spastic paraparesis are caused by htlv2. It is caused by the human tcell lymphotropic virus1 htlv1 retrovirus. Tropical spastic paraparesis in kerala, south india oomman a. 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. 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 paraparesis brain, spinal cord, and.
Its caused by the human tcell lymphotrophic virus type. Bruhn, in mandell, douglas, and bennetts principles and practice of infectious diseases eighth edition, 2015. Spastic paraplegia type 4 is a pure hereditary spastic paraplegia. Common and important causes of spastic paraparesis for doctors and medical students this page is currently being written and will be available soon. Idiopathic progressive spastic paraparesis jama jama. Those areas were found to be endemic for the htlvi, the etiologic agent for hamtsp. Early in the disease course, there may be mild gait difficulties and stiffness.
A case study r narismulu greys hospital february 2008. Tropical spastic paraparesis definition tropical spastic paraparesis tsp is an incurable viral infection of the spinal cord that causes weakness in the legs. Possible etiologies for tropical spastic paraparesis and. Hereditary spastic paraplegia is a general term for a group of rare inherited disorders that cause weakness and stiffness in the leg muscles. Htlv1 proviral load in infective dermatitis associated with. Hereditary spastic paraparesis affects both sexes and may begin at any age. Adultonset, chronic progressive spastic paraparesis may be due to a large number of causes and poses a diagnostic challenge. 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. Tropical spastic paraparesis, human tcell lymphotropic virus.
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. Hereditary spastic paraparesis hsp is a heterogeneous group of inherited disorders, with prevalence ranged from 4. Paraplegia is an impairment in motor or sensory function of the lower extremities. In 1993, htlvii was isolated in the absence of any other detectable human retroviruses from a.
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. Hereditary spastic paraplegia genetic and rare diseases. Hereditary spastic paraplegia hsp is a group of hereditary, degenerative, neurological disorders that primarily affect the upper motor neurons. Multiple sclerosis, tropical spastic paraparesis and journal of. Hereditary spastic paraplegia information page national. 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. Tropical spastic paraparesis tsp, is a medical condition that causes weakness, muscle spasms, and sensory disturbance by human tlymphotropic virus resulting in paraparesis, weakness of the legs. All forms cause degeneration of the nerve pathways that carry signals from the brain down the spinal cord to muscles. Upper motor neurons in the brain and spinal cord deliver signals to the lower motor neurons, which in turn, carry messages to the muscles. Treatment is symptomatic, including drugs to relieve spasticity.
It is usually caused by spinal cord injury or a congenital condition that affects the neural brain elements of the spinal canal. 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 is listed as a rare disease by the office of rare diseases ord of the national institutes of health nih. Please include your name and address, but these will be omitted on request. Hereditary spastic paraplegia hsp is a group of rare, inherited neurological disorders. Current neurology and neuroscience reports, 2006, 61. People with this condition can also experience exaggerated reflexes hyperreflexia, ankle spasms, higharched feet pes cavus, and reduced bladder. 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 is a group of rare hereditary disorders characterized by progressive, spinal, nonsegmental spastic leg paresis, sometimes with intellectual disability, seizures, and other extraspinal deficits. Tropical spastic paraparesis definition of tropical. Hereditary spastic paraplegia knowledge for medical.
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. Tropical spastic paraparesis an overview sciencedirect. Hsp is caused by defects in genes which code for proteins essential for fast axonal neurotransmission. Proposal for diagnostic criteria of tropical spastic paraparesishtlvi associated myelopathy tspham. 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. Lecturer, alvas college of physiotherapy, moodabidri, india1. Tropical spastic paraparesis tsp is a chronic degenerative neurological disease associated with this infection. There are two forms of paraparesis which further explain the primary causes of the disease.
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