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Postpump chorea (PPC) is the development of choreoathetoid movements within 2 weeks following cardiopulmonary bypass. Over a 10-year period, 668 children underwent open cardiac surgery, of whom 8 (1.2%) developed PPC. Age at surgery ranged from 8 to 34 months. The onset of chorea was 3 to 12 days following surgery. Computed tomography and magnetic resonance imaging showed atrophy but no focal lesions. Cerebral positron emission tomography using [18F]fluorodeoxyglucose in a patient following 12 months of chorea showed patchy areas of decreased glucose metabolism. None of the patients were developmentally normal 22 to 130 months following surgery. Three patients have had transient and 5 have persistent chorea. Neurological deficits ranged from a mild learning disability to progressive hypotonia and obtundation ending in death. One of 4 patients who received haloperidol had a decrease in the severity of chorea. We compared PPC patients with 39 randomly selected controls. During surgery, affected patients spent significantly more time on pump and at temperatures under 36 degrees C, were cooled to lower temperatures than controls, and were more likely to have had a circulatory arrest. One patient developed chorea without a history of circulatory arrest. We conclude that (1) there is a strong association between PPC, deep hypothermia, and circulatory arrest, (2) absence of characteristic macroscopic changes suggests a biochemical or microembolic etiology in some cases, (3) chorea is frequently associated with developmental delay, and (4) the prognosis for complete resolution of chorea is guarded.

Four patients presented with hemiballism-hemichorea as a clinical manifestation of white matter ischemia. These patients illustrate "positive" motor phenomena rather than limb weakness as a consequence of cerebral ischemia. In each patient, the involuntary movements disappeared following worsening of paresis. Subcortical white matter infarction in three patients and hemodynamic hypo-perfusion in the cerebral hemisphere contralateral to dyskinetic movements were possible causes. Neuroradiologically, none had pathological changes in the vicinity of the subthalamic nucleus. We presume from these observations that ischemia of the subcortical white matter, without involvement of the basal ganglia or the subthalamic nucleus, may cause hemiballism-hemichorea.

Detailed clinical and neuropathological findings in two unrelated patients with a chorea-acanthocytosis-like phenotype (CA) are reported. One case met all the diagnostic criteria of CA and had a deceased brother with the same disease. The second case had a virtually identical phenotype to the former but without acanthocytes. These findings suggest that both patients are affected by the same disease and that acanthocytes are not essential to the diagnosis. Neuropathological autopsy studies on the brain of the second case showed selective atrophy of the caudate nucleus that seemed to correspond to the movement disorder and behavioural abnormalities prominent in this patient. In both subjects, morphometric and ultrastructural examination of the peripheral nerve showed loss of myelinated fibres, more accentuated distally, and cytoskeletal changes in the axoplasm. These findings support the hypothesis that peripheral neuropathy in CA is caused by distal axonopathy.

Patients with chorea-acanthocytosis exhibit symptoms of self-biting, choreic movement, and acanthocytosis, but not dementia. The mechanism of choreic movements is still unknown. In order to clarify the etiologic mechanism underlying these movements, we evaluated the erythrocyte membrane in one patient with chorea-acanthocytosis. A 35-year-old female was admitted to Saitama Medical School Hospital because of involuntary movements. She was alert, well-oriented, and had no gross memory defects. She had slurred speech, choreic movements and lip biting. Laboratory examination showed acanthocytes in her peripheral red blood cells, normal serum lipid values, and caudate atrophy on her brain CT scan. In analyzing the acanthocytes, we initially evaluated the size of the acanthocyte population by incubating her red blood cells with plasma. The cell population approximately doubled after 2 hours incubation. Next we examined the protein composition of erythrocyte ghost by sodium dodecyl sulfate polyacrylamide gel electrophoresis (SDS-PAGE). There was no significant difference between the patient's erythrocyte ghosts and those of a control. Then we investigated morphological changes in the patient's erythrocyte by scanning and transmission electron microscopy (SEM and TEM). SEM showed the typical acanthocyte shape. The quick-freeze, freeze-substitution method confirmed that the routine TEM section was not artifactual, and was in fact in accurate reflection of the actual features of acanthocytes. TEM of the sections prepared from erythrocyte ghosts demonstrated that spectrin tended to be accumulated in the thorn region. Furthermore, TEM of quick-freeze, deep-etched replica of the ghost revealed more clearly a spectrin network densely packed on the inner hydrophilic surface.(ABSTRACT TRUNCATED AT 250 WORDS)

Dyskinesias associated with dopaminergic treatment in idiopathic Parkinson's disease (PD) can be indistinguishable from those arising spontaneously in other conditions involving degeneration of, or damage to, the basal ganglia. However, those due to levodopa treatment of PD disappear on cessation of therapy. We report a patient with a clinical diagnosis of PD who, on levodopa treatment, developed hemiballism and chorea that were originally thought to be drug induced. However, the dyskinesias persisted despite stopping levodopa. Postmortem analysis showed a multisystem degeneration.

Two patients with striatal atrophy and a clinical syndrome consistent with choreoacanthocytosis had normal dried blood smears but their red cells demonstrated an abnormal sensitivity to various conditions known to promote discocyte-echinocyte transformation. Dilution in normal saline, in vitro aging, and contact with glass caused a great proportion of these patients' red cells to develop multiple spiny or rounded projections. Under identical conditions, such shape changes did not occur in normal patients or in those with Huntington's disease. Scanning electron microscopy showed that the age-induced increase in acanthocytic-appearing cells could be reversed with chlorpromazine. These data suggest that the red cells from these patients with striatal degeneration are deficient in their ability to preserve normal shape in the face of echinocytic stress and that this observation has diagnostic and, possibly, pathophysiologic significance.

Chorea has been related to the presence of antiphospholipid antibodies (a-PL) in the context of systemic lupus erythematosus (SLE). Here we report the case of a 13-year-old girl with a-PL antibodies, who had developed thrombophlebitis at the age of 11 years and chorea two years later, in the absence of clinically evident SLE. Serological tests revealed a false positive test for syphilis, a prolonged activated partial thromboplastin time, hypocomplementaemia and positive anti-DNA antibodies.

This report describes a case of central pontine myelinolysis occurring after a rapid correction of profound hyponatremia. Delayed-onset generalized dystonia and choreoathetosis then appeared. A small pontine myelinolysis was demonstrated by magnetic resonance images, but striatal myelinolysis could not be established. Aspects of movement disorders associated with the osmotic demyelination syndrome are briefly reviewed and discussed.

Neuropeptide immunohistochemistry was used to test several hypotheses of the anatomical bases of chorea and rigidity-akinesia. To test the hypothesis that elevated concentration of striatal somatostatin causes chorea, we visually compared the density of striatal neurons containing somatostatin and neuropeptide Y in brains affected by choreic or rigid-akinetic Huntington's disease (HD). The density of these neurons was elevated in both rigid-akinetic and choreic HD specimens with an apparently normal total number of these neurons, indicating that elevated somatostatin concentration, by itself, does not lead to chorea. We tested the hypothesis that rigid-akinetic HD results from deficient dopaminergic nigrostriatal neurotransmission by examining tyrosine hydroxylase-immunoreactive (TH-IR) neurons in the substantia nigra. In rigid-akinetic HD brains, there was no obvious reduction of nigral TH-IR neurons, indicating that rigid-akinetic HD is probably not due to loss of nigral dopaminergic neurons. Finally, we also examined the status of striatal projection neurons and found near total loss of all striatal neurons projecting to the lateral globus pallidus, medial globus pallidus, and substantia nigra in brains affected by rigid-akinetic HD in contrast to the preservation of neurons projecting to the medial globus pallidus in choreic HD. These results are consistent with the hypothesis that chorea results from preferential loss of striatal neurons projecting to the lateral globus pallidus and that rigid-akinetic HD is a consequence of the additional loss of striatal neurons projecting to the medial segment of the pallidum.

In order to produce choreic movements in monkeys (Macaca species), we have used the combination of unilateral kainic acid injections into the striatum and the systemic administration of L-DOPA. Two control monkeys received kainic acid injections alone and 7 were also given L-DOPA (150-300 mg/day) orally. No spontaneous involuntary movements were seen after kainic acid injection alone (over a period of 4-14 days). In the 7 monkeys that received L-DOPA 4-7 days after the kainic acid injection, in 5, whose striatal lesions were located in the rostral dorsolateral striatum, choreic movements appeared in the contralateral limbs following each dose of L-DOPA, whereas in 2 monkeys in which the lesion was large enough to occupy more than 60% of the rostral striatum, no choreic movements occurred. Preservation of some part of the striatum is therefore necessary for the generation of choreic movements. A considerable number of neurons in the rostral ventromedial putamen, which escaped the kainic acid lesion showed burst discharges when L-DOPA was administered. These were not seen in the caudate nucleus. The burst discharges recorded from some neurons were time-locked to the choreic movements. Since deafferentiation by dorsal root section had no effect, these burst discharges appeared to be generated in the unlesioned striatum during the choreic movements. Brains of 6 monkeys were analysed biochemically for gamma-aminobutyric acid concentration, choline acetyltransferase and tyrosine hydroxylase activity, and spiroperidol binding. Concentrations of gamma-aminobutyric acid, choline acetyltransferase activity and spiroperidol binding in the basal ganglia did not correlate with the generation of choreic movements. A finding which appeared to have a clear correlation was an increase in excess of 50% in tyrosine hydroxylase activity in the rostral ventromedial striatum on the lesioned side which escaped the kainic acid lesion in choreic monkeys as compared with the corresponding area on the intact side. It is, therefore, assumed that certain populations of 'activated' nigrostriatal dopaminergic neurons which innervate the unlesioned area of the striatum may be more likely to attain a level of activity, induced by L-DOPA, sufficient to produce choreic movements in the contralateral limbs. Methamphetamine, but not apomorphine, given to 2 monkeys, produced an effect that was essentially the same as that of L-DOPA. These results support the hypothesis that the 'activated' dopaminergic components in choreic monkeys are the presynaptic dopaminergic nerve terminals rather than the postsynaptic dopaminergic receptors.

An unusual case of severe hemichorea in an elderly hypertensive woman, precipitating acute pulmonary edema, is presented. There were small cystic infarctions in the contralateral neostriatum and thalamus, and intact subthalamic nuclei. The clinicopathologic correlation of hemichorea and hemiballismus is reviewed, and its relationship with the results of recent neuroanatomic research is discussed.

We report a case of choreo-acanthocytosis in a 33 year-old man with mild chorea of the limbs but no involuntary movements of the face, areflexia, epilepsy and personality changes. Acanthocytosis was 10-20% and creatine-phosphokinase levels were raised. Electrophysiological data were consistent with lower motor neurone dysfunction. Muscle biopsy revealed changes typical of neurogenic atrophy. Nerve biopsy showed a severe loss of large myelinated axons. Electron microscopic examination was in favour of primary axonal damage affecting mainly myelinated fibers and only slightly unmyelinated fibers.

Senile chorea, a late-onset, slowly progressive disorder without mental changes, is a poorly characterized entity. It is often thought to represent late-onset Huntington's disease in which the chorea precedes the mental changes and the family history is absent due to parental death before symptoms. The following case differs pathologically from Huntington's disease and from previous reports of senile chorea.

A case with choreoathetoid syndrome is described in a patient with advanced picture of systemic lupus erythematosus. The ineffective cerebroprotective therapy is associated with the grave affection of the deep cerebral structures by he lupus vascuitis. The adequate interpretation of the pathological deviations in the CNS in systemic lupus could contribute to their early diagnosis and timely treatment.

In a 60-year man with a very slowly progressive motor disturbance probably of torsion systonic or choreoathetotic type which started in childhood, we found an isolated degeneration of the exterior pallidum with intraneuronal deposition of Bielschowsky bodies and only slight deficit of neurons. The Bielschowsky bodies show the same structure as corpora amylacea and Lafora bodies. They might also be the result of a cellular disturbance mainly concerning the carbohydrate metabolism. In this case they are localised only in the neurons of the exterior part of the pallidum--in contrast to the organogenitically otherwise deriving (from the nucl. entopeduncularis) intact neurons of the pallidum internum--and they induce, after an excessive accumulation of the metabolites, not till extremely late loss of neurons. This circumstance explains the very torpid progress of this disease.

In three cases of chorea-acanthocytosis (acanthocytosis and neurological disease, or familial degeneration of the basal ganglia with acanthocytosis), biopsies of short peroneal muscles and sural nerves were studied histologically. The muscles showed groups of atrophic fibres with clumping of sarcolemmal nuclei in all cases. It was concluded that neurogenic muscular atrophy should be included as one of the main pathological findings in chorea-acanthocytosis. The sural nerves showed a small number of large myelinated fibres in two cases. This finding remains to be confirmed in other cases.

We investigated involvement of the peripheral nervous system in 6 patients with amyotrophic chorea-acanthocytosis. Electromyographic and neurographic findings, and pathological changes as demonstrated by examination of biopsy specimens of muscle and sural nerve indicate that most patients had an axonal sensorimotor polyneuropathy with more pronounced involvement of the distal portion of the nerves. Results obtained in one patient raised the question of an anterior horn cell disorder.

In a 23-year-old primigravida with no prior history of rheumatic fever, choreiform movements developed late in the third trimester, and she died in a state of hyperthermia 14 days later. Results of neuropathological examination showed, as the basis for the chorea, nerve cell loss and astrocytosis within the striatum (especially the caudate nucleus). Anaylsis of the case in conjunction with a review of the literature strongly suggests that chorea gravidarum should be regarded as a syndrome rather than a specific disease entity.

Chorea-acanthocytosis has been separated as a clinical entity different from Huntington's chorea, mainly based on the clinical findings, but the histopathological and biochemical features of chorea-acanthocytosis, especially of basal ganglia, have not been well established, because only two such autopsy cases have been reported. The case presented here was a 39-year-old man at autopsy, with 10 years duration of typical symptoms and signs of chorea-acanthocytosis. At autopsy, the abnormal histopathological findings in the central nervous system were mainly confined to the striatum, where the caudate nucleus and putamen showed severe and moderate atrophy, respectively. Morphometric evaluation of the numbers of small and large neurons in the striatum with the adjustment for the shrinkage produced in the disease processes was performed. The numbers of small neurons in the caudate nucleus and putamen were 1% and 20% of each control, respectively. On the other hand, the large neurons in the caudate nucleus showed a reduction of diameters without a decrease in number and those in the putamen showed a mild decrease in number. In the biochemical studies, marked decrease of substance P (SP) level without definite decrease of choline acetyltransferase and glutamic acid decarboxylase (GAD) activities in both caudate nucleus and putamen was found. Substantia nigra, where no evident histopathological abnormalities were found, showed definite decrease of GAD activity and SP level. In the peripheral nervous system, the lateral branch of deep peroneal nerve showed mild degree of axonal degeneration. Neurogenic muscular atrophy with severe and mild degrees was found in extensor digitorum brevis and quadriceps femoris muscles, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

Antagonists of the neurotransmitter gamma aminobutyric acid (GABA) were injected at seventy intracerebral sites in the conscious monkey. Injections located in or near to the subthalamic nucleus provoked involuntary movements of the contralateral limbs. These movements had the characteristics of chorea and, when severe, resembled hemiballismus as seen following subthalamic nucleus lesions in man and monkey. Localized injections of GABA antagonists into the medial segment of the globus pallidus were without observable effect on motor activity. These observations are discussed with regard to the role of GABA in subthalamopallidal relations. Injection of GABA antagonists at some other sites provoked different forms of dyskinesia. Injections in or near the zona incerta adjacent to the subthalamic nucleus induced torticollis and circling behaviour either in isolation or in addition to contralateral limb dyskinesia. Injections at some sites located in the lateral segment of the globus pallidus or in the putamen induced choreoathetoid or myoclonic movements, respectively.

The striatum, pallidum and subthalamic nucleus were studied by combined morphometric methods in serial sections of 13 brains of normal adults and of 15 patients with choreatic diseases. In addition the volume of the hemispheres and of the cortex were measured. All data obtained were corrected by the shrinkage factor to represent fresh brain values. In Huntington's chorea the pallidum was more severely affected than is commonly appreciated. The average volume reduction was of the same degree (lateral-57%, medial-50%) as that of the striatum (-56%). The absolute number of nerve cells of the pallidum decreased in both segments by about 40%. The reduction of the volume and of the number of nerve cells was not reduced in the three subcortical nuclei studied. For the first time it has been shown that there is no increase in the absolute number of glial cells in the striatum. The increased numerical density of glial cells is caused by shrinkage. The loss of nerve cells of the pallidum and subthalamic nucleus is caused mainly by a primary process. Huntington's chorea is a multifocal process. Morphometric data do not suggest that subchorea is a variant of Huntington's chorea. Chorea minor is regarded as a multifocal process with varying affliction of the striatum, pallidum and subthalamic nucleus. An increase in the number of glial cells and, as a rule, a moderate loss of nerve cells were found in this disease.

An analysis is presented of 35 cases of chorea as a symptom of polycythaemia. This analysis reveals: (a) whereas polycythaemia occurs predominantly in males (3:2), polycythaemic chorea (PC) occurs predominantly in females (5:2), at a real ratio of female:male = 4:1, the prevalence being 1-2.5% of polycythaemic patients; (b) PC manifests predominantly after the age of 50 (8 cases before, 27 after 50 years), making polycythaemia the first disorder to be considered in cases of so-called 'senile' chorea; (c) PC is generalised, with predominant involvement of faciolingual and brachial muscles, and associated with muscular hypotonia; (d) PC may last from periods of weeks to years, usually responds to haloperidol, venesection or 32P-treatment, but may persist, or recur with treatment, or remit spontaneously, and (e) no relationship exists between the choreatic syndrome and (the rare finding of) a small infarct in the caudate nucleus. The cause of the choreatic syndrome in polycythaemia is presumably to be explained as a neostriatal hyperviscosity syndrome producing venous stasis, reduced brain blood flow and impaired tissular O2/glucose metabolism. The state of dopaminergic hyperactivity is presumably enhanced by relatively increased neostriatal catecholestrogens. The hypothesis of polycythaemic excess of dopamine-laden platelets releasing excess of dopamine in the neostriatum needs to be confirmed by laboratory evidence of platelet counts.

We performed a neurochemical study of the brain of two unrelated patients, living in different continents, with neuroacanthocytosis. The levels of monoamines and their metabolites, gamma-aminobutyric acid and substance P, were measured in several brain areas and the monoamine metabolites in cerebrospinal fluid. The binding of 3H-spiperone to striatal membranes and to lymphocytes was also measured. Both patients had a progressive neurological disorder with onset in the third decade of life and characterized by a complex movement disorder, epilepsy, muscular wasting, and changes in behavior. The movement disorder initially manifested with oromandibular dystonia and limb chorea, but at the time of death was characterized by a severe dystonic syndrome. The chemical changes were similar in the two patients. The most important neurochemical findings were a depletion of dopamine and its metabolites in most brain areas, most notably in the striatum, and elevation of norepinephrine levels in the putamen and globus pallidus. Substance P was markedly reduced in the striatum and substantia nigra. Our findings may provide clues to the neurochemical mechanisms underlying dystonia.

A 45-year-old woman with an acquired multifocal neurologic syndrome, including chorea, dystonia, cerebellar dysfunction, multiple cranial neuropathies, and pure sensory neuropathy, was found at autopsy to have oat cell carcinoma. Neuropathologic examination revealed several features typically associated with remote effects of malignancy on the nervous system. We believe that this is the first described case of chorea as a remote effect of malignancy.

Neuropathological studies of a woman with chorea-acanthocytosis were reported. The patient clinically showed generalized epileptic seizures, choreatic involuntary movements, hypotonia , areflexia, neurogenic muscular atrophy, tongue-biting and acanthocytosis of the peripheral blood and died from asphyxia at the age of 34. Autopsy revealed atrophy of the caudate nucleus but the cerebral cortex was well preserved. Histological examination showed severe neuronal loss associated with moderate fibrous gliosis of the caudate. The putamen was similarly but less markedly affected. The small striatal neurons were more severely depopulated than the large neurons, although the latter were not spared. Cytometrical study of the caudate head revealed that the remaining small neurons were significantly larger in size than the normal small neurons of the same area. The anatomical substratum of the choreatic involuntary movements in chorea-acanthocytosis is thought to be the degeneration of the striatum, especially the caudate nucleus. Neuropathological differentiation of chorea-acanthocytosis from Huntington's chorea which shows similar pathology was discussed.

A critical analysis of 52 cases of systemic lupus erythematosus (SLE) and chorea is presented. Choreatic movements occurred early in the course of the disease in most cases; the duration varied from several days to 3 years. No sex predominance was found and no relationship could be demonstrated between chorea and other neurological signs, or between chorea and other SLE manifestations. Also, the neuropathological findings offered no simple explanation for the emergence of chorea. Immunological pathomechanisms might be present.

Five generations of a family exhibit a unique autosomal dominant neurologic disorder characterized by the development (usually between 15 and 30 years of age) of ataxia, seizures, choreiform movements, progressive dementia, and death after 15 to 25 years of illness. Neuropathologic findings in two deceased family members revealed remarkably similar findings, including marked neuronal loss of the dentate nucleus, microcalcification of the globus pallidus, neuroaxonal dystrophy of the nucleus gracilis, and demyelination of the centrum semiovale. The clinical and pathologic findings are closely correlated. Ataxia and chorea are related to severe neuronal loss in the dentate nucleus with calcification in the globus pallidus. Dementia occurs with progressive demyelination of the centrum semiovale, and loss of posterior column function occurs with neuroaxonal dystrophy of the nucleus gracilis and nucleus cuneatus.

The clinical picture of Morvan's fibrillary chorea includes a. spontaneous muscular activity resulting from repetitive motor unit action potentials of peripheral origin (multiplets), b. autonomic dysregulation with profuse hyperhidrosis, and c. central nervous system involvement as shown by severe insomnia and hallucinosis. A case featuring all these symptoms is presented. Whereas known causative factors range from gold or mercury poisoning to autoimmune disorders, the presented case is the first one in which chronic manganese intoxication (occupational exposure) seems to be implicated. Manganese has been found to inhibit acetylcholine esterase, and, as a consequence, may produce peripheral and central cholinergic hyperactivity.

We studied two siblings with chorea and amyotrophy of adult onset and spherocytic hemolytic anemia. Autopsy revealed an atrophic striatum with iron deposition and spheroid bodies. Degeneration of the substantia nigra and spinal cord anterior horns was seen without iron deposition. The disorder seems to be a variant of the chorea-amyotrophy-acanthocytosis syndrome, and the pathology may account for evidence of parkinsonism and amyotrophy in that syndrome. Acanthocytosis may not be the only hematologic abnormality in this disorder.

A critical analysis of cases of systemic lupus erythematosus (SLE) and chorea is presented. Choreatic movements occurred early in the course of the disease in most cases; the duration varied from several days to 3 years. No sex predominance was found and no relationship could be demonstrated between chorea and other neurological signs, or between chorea and other SLE manifestations. Also, the neuropathological findings offered no simple explanation for the emergence of chorea. Immunological pathomechanisms might be present.

A giant fisuform basilar artery aneurysm ruptured, causing the death of an 11-year-old girl who had presented with a 5-month history of headaches and a 1-month history of progressive brainstem features with choreiform movements. This case, unlike other reported cases, demonstrated no evidence of a generalized arteriopathy, and draws attention to the occurrence of this rare cause in the differential diagnosis of progressive brainstem syndrome in children.

The finding in a 40-year old and apparently healthy woman of a pseudotumoral lesion of the left arm which had gradually developed over a 9-year period led to a tentative clinical diagnosis of lupus anetoderma. This diagnosis was confirmed on the one hand by histological examination of the skin--which showed almost total disappearance of dermal elastic fibres, while the lympho-histiocytic infiltrate was so discreet that it excluded a deep lupus--and on the other hand by direct immunofluorescence, with lupus band on the lesion and a few abnormalities of the exposed and covered healthy skin. A history of chorea at the age of 13 years raised the problem of a possible relationship between this neurological disease and a systemic lupus erythematosus. This hypothesis was supported by resolutive episodes of arthralgia and abnormal laboratory findings, such as accelerated ESR, leucopenia, decrease of complement and presence of homogeneous antinuclear antibodies. Anticardiolipin antibodies, circulating anticoagulants and VDRL test were negative. Treatment with anti-malarials brought about some degree of cutaneous shrinkage and a significant decrease in ESR. This case is reported because anetoderma as sole manifestation of systemic lupus erythematosus and the occurrence, 17 years previously, of chorea are exceptional events.

Clinical and neuropathological descriptions are given of four cases of an uncommon disease, characterised by simultaneous degeneration of the dentato-rubral and pallido-luysian systems. These four are compared with sixteen previously described cases, and the group as a whole is compared and contrasted with other multisystem degenerations, such as olivo-ponto-cerebellar atrophy and Friedreich's ataxia. A pathological feature described here for the first time is degeneration of the fastigio-vestibular system. Clinically, there are three main types of the disease; (1) an ataxo-choreoathetoid type, (2) a pseudo-Huntington type, and (3) a myoclonic-epileptic type. There are familial cases of types 2 and 3. Oculomotor disturbances, associated with atrophy of the brainstem tegmentum, are observed in cases of types 1 and 3.

Olivopontocerebellar atrophy is a hereditary disorder that has variable clinical manifestations. Five types have been described, as well as a sixth that contains sporadic cases. This report describes a family with three affected members who demonstrate a composite of types III and V. Their features include progressive spasticity, ataxia, dementia, visual loss with retinal pigmentation, dysarthria, ophthalmoplegia, and chorea. This family might represent an additional category of the disease. In the two family members who developed chorea, baclofen resulted in marked improvement with abolition of the choreiform movements. Response has been sustained for several years in the mother and for eight months in the daughter. Neither has experienced any return of chorea while receiving treatment. When attempts were made to discontinue baclofen, choreiform movements returned promptly and with their original severity. Baclofen, a gamma-aminobutyric acid analogue, may be useful in the treatment of other forms of chorea as well.

A familial disorder was characterized by chorea, ataxia, myoclonus, convulsions, dementia, and mental retardation. In five cases, the main lesion affected cerebellar dentate nuclei, with nerve cell loss, gliosis, chromatolysis, and grumose degeneration. Fibrous glial cell proliferation was detected in the globus pallidus.