Huntington's Chorea, Sydenham's Chorea (Rheumatic chorea, St.
Vitus dance), Kernicterus and Neurological involvement in Wilson's disease (Hepatolenticular degeneration) are all typical types of primary involuntary movements and diseases of the extrapyramidal system.
Their seizure activities may take different patterns and hence a need to understand each and every one of them.
Huntington's Chorea This is a degenerative disease of the central nervous system, inherited as an autosomal dominant, clinically characterized by dementia and choreoathetosis.
Males are affected more.
The clinical manifestations start by the fourth of fifth decade, though in some cases symptoms start early.
Pathologically, the lesion consists of gross strophy of the caudate nucleus and the putamen bilaterally.
The gyri of the frontal and temporal cortex also show atrophy.
Clinical features: The clinical features vary in severity.
Combinations of psychiatric disturbances and choreoathetetic movements are seen.
The mental changes may range from mild impairment of higher functions to dementia.
The abnormal movements consist of restlessness, fidgetiness and choreoathetosis involving the extremities and trunk.
The psychiatric and neurological manifestations often coexist or either may set in earlier.
With successive generations, the disease manifests in younger ages (anticipation).
The disease progresses relentlessly over 10-15 years to end fatally.
Diagnosis is clinical-it is easy in the fully developed form.
In those with incomplete manifestation, presence of the disease in family members is a helpful diagnostic feature.
The Corpus striatum is more sensitive to dopamine in Huntington's chorea and therefore L-dopa brings our the abnormal movements and aggravates them.
In those with subclinical manifestations, this can be used for diagnosis.
CT Scan helps in visualizing the cerebral atrophy and dilatation of the anterior horns of the lateral ventricles.
Treatment: Early cases may benefit by phenothiazines (e.
g, fluphenazime) and butyrophenones (e.
g, haloperidol).
Anti-parkinsonism drugs may help in some cases but L-dopa has to be avoided.
Sydenham's Chorea (Rheumatic Chorea, St.
Vitus dance) This is one of the acute rheumatic manifestations.
Pathological changes consists of edema and congestion of different regions of the brain.
Microscopy shows marked changes in the Corpus striatum, subthalamic nuclei, substantia nigra, and cerebral cortex.
The acute changes consist of vasodilatation and perivascular degeneration.
Neuronal loss and perivascular glial proliferation have been described.
Adolescent girls are affected more often.
The onset is abrupt with generalized chorea and emotional liability.
Volitional activity and emotional excitement worsen the abnormal movements.
Most of the cases recover fully even without treatment.
Haloperidol and diazepam give symptomatic relief of the abnormal movements.
Kernicterus This is a sequel to severe neonatal jaundice occurring in Rh incompatibility or ABO hemolytic disease.
The unconjugated bilirubin crosses the blood brain barrier and depresses oxidative metabolism of brain cells, especially the basal ganglia.
Initial symptoms include convulsions, Opisthotonus, rigidity, and coma.
Late sequelae are athetoid movements, spasticity, nerve deafness, and mental deficiency, which manifest by the age of 6-7 months.
The pathological changes include loss of neurons and gliosis in the subthalamic nucleus, thalamus, globus pallidus and the nuclei of the oculomotor and cochlear nerves.
Once established, the condition tends to be permanent.
Early detection of neonatal jaundice and treatment by exchange transfusion are important prophylactic measure to avoid neurological damage.
Neurological involvement in Wilson's disease (Hepatolenticular degeneration) In the brain, the basal ganglia, cerebellum and cerebral cortex show deposition of copper.
The clinical manifestations are involuntary movements which consists of fine and coarse tremors.
Vitus dance), Kernicterus and Neurological involvement in Wilson's disease (Hepatolenticular degeneration) are all typical types of primary involuntary movements and diseases of the extrapyramidal system.
Their seizure activities may take different patterns and hence a need to understand each and every one of them.
Huntington's Chorea This is a degenerative disease of the central nervous system, inherited as an autosomal dominant, clinically characterized by dementia and choreoathetosis.
Males are affected more.
The clinical manifestations start by the fourth of fifth decade, though in some cases symptoms start early.
Pathologically, the lesion consists of gross strophy of the caudate nucleus and the putamen bilaterally.
The gyri of the frontal and temporal cortex also show atrophy.
Clinical features: The clinical features vary in severity.
Combinations of psychiatric disturbances and choreoathetetic movements are seen.
The mental changes may range from mild impairment of higher functions to dementia.
The abnormal movements consist of restlessness, fidgetiness and choreoathetosis involving the extremities and trunk.
The psychiatric and neurological manifestations often coexist or either may set in earlier.
With successive generations, the disease manifests in younger ages (anticipation).
The disease progresses relentlessly over 10-15 years to end fatally.
Diagnosis is clinical-it is easy in the fully developed form.
In those with incomplete manifestation, presence of the disease in family members is a helpful diagnostic feature.
The Corpus striatum is more sensitive to dopamine in Huntington's chorea and therefore L-dopa brings our the abnormal movements and aggravates them.
In those with subclinical manifestations, this can be used for diagnosis.
CT Scan helps in visualizing the cerebral atrophy and dilatation of the anterior horns of the lateral ventricles.
Treatment: Early cases may benefit by phenothiazines (e.
g, fluphenazime) and butyrophenones (e.
g, haloperidol).
Anti-parkinsonism drugs may help in some cases but L-dopa has to be avoided.
Sydenham's Chorea (Rheumatic Chorea, St.
Vitus dance) This is one of the acute rheumatic manifestations.
Pathological changes consists of edema and congestion of different regions of the brain.
Microscopy shows marked changes in the Corpus striatum, subthalamic nuclei, substantia nigra, and cerebral cortex.
The acute changes consist of vasodilatation and perivascular degeneration.
Neuronal loss and perivascular glial proliferation have been described.
Adolescent girls are affected more often.
The onset is abrupt with generalized chorea and emotional liability.
Volitional activity and emotional excitement worsen the abnormal movements.
Most of the cases recover fully even without treatment.
Haloperidol and diazepam give symptomatic relief of the abnormal movements.
Kernicterus This is a sequel to severe neonatal jaundice occurring in Rh incompatibility or ABO hemolytic disease.
The unconjugated bilirubin crosses the blood brain barrier and depresses oxidative metabolism of brain cells, especially the basal ganglia.
Initial symptoms include convulsions, Opisthotonus, rigidity, and coma.
Late sequelae are athetoid movements, spasticity, nerve deafness, and mental deficiency, which manifest by the age of 6-7 months.
The pathological changes include loss of neurons and gliosis in the subthalamic nucleus, thalamus, globus pallidus and the nuclei of the oculomotor and cochlear nerves.
Once established, the condition tends to be permanent.
Early detection of neonatal jaundice and treatment by exchange transfusion are important prophylactic measure to avoid neurological damage.
Neurological involvement in Wilson's disease (Hepatolenticular degeneration) In the brain, the basal ganglia, cerebellum and cerebral cortex show deposition of copper.
The clinical manifestations are involuntary movements which consists of fine and coarse tremors.