Hereditary spastic paraplegia hsp is a group of hereditary, degenerative, neurological disorders that primarily affect the upper motor neurons. Cervical myelopathy c5t1 anterior horn cell disease motor neuron disease syringomyelia. Hereditary spastic paraplegia hsp is a group of neu rodegenerative monogenic disorders characterized by dysfunction of the longer tracts of the spinal cord. 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 hsp, spg loci, strumpelllorrain syndrome, pure and complex forms. Here we present, for the first time we believe, the neuropathological findings in an hsp type. Lorrain disease, is a relatively rare inherited neurodegenerative syndrome of the central nervous system and. Pdf hereditary spastic paraplegia comprises a wide and heterogeneous group of inherited neurodegenerative and neurodevelopmental. Tropical spastic paraparesis tsp tsp is a disease of the nervous system that causes weakness, stiffness, and muscle spasms of the legs.
Hereditary spastic paraplegia, axonal degeneration, pure. From the epidemiological studies, the prevalence rate ranges between 4. Chapter differential diagnosis of acquired causes of spastic. Spastic paraplegia reveals in most cases a disease affecting the spinal cord. Hereditary spastic paraparesis hsp or the strumpelllorrain syndrome is the name given to the rare inherited autosomal dominant disorders in which the main clinical feature is progressive lower limb spasticity.
Adultonset spastic paraparesis journal of the neurological sciences. Clinical features and management of hereditary spastic paraplegia. 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 limb spasticity. Syndrome ofthe month pure hereditary spastic paraplegia ncbi. Diagnosis, investigation and management of hereditary spastic. Case report physical therapy interventions for the. Spastic paraplegia 4 spg4 is the most common type of hereditary spastic paraplegia hsp inherited in an autosomal dominant manner.
Adultonset, chronic progressive spastic paraparesis may be due to a large number of causes and poses a diagnostic challenge. Tropical spastic paraparesis tropical spastic paraparesis tsp is an endemic neurological disorder found in many tropical and subtropical areas, such as the caribbean islands, southern united states, south america, and africa. Hsps are a group of genetic conditions in which spastic paralysis of. Hereditary spastic paraplegia hsp is a group of geneticallydetermined disorders characterized by progressive spasticity and weakness of lower limbs. Hereditary spastic paraplegia genetic and rare diseases. Pdf hereditary spastic paraparesis hsp or the strumpelllorrain syndrome is the name given to a heterogeneous group of inherited disorders in which. Tropical spastic paraparesis an overview sciencedirect. No treatment is known to reduce disease progression, but antispastic. International retrovirology association guidelines for the. Spg4 is characterized by slowly progressive muscle weakness and spasticity stiff or rigid muscles in the lower half of the body.
Gait patterns in patients with hereditary spastic paraparesis. Pure hereditary spastic paraplegia is the most common form of hereditary spastic paraplegia. One of the most abundant fad mutations, psen1 a431e, has been reported to be associated with spastic paraparesis in. Hereditary spastic paraplegia type 8 neuropathological.
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