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Sydenham's chorea is the most common acquired cause of chorea in the third world. We report a case of recurrent chorea successfully treated with sodium valproate. Miss A.C, aged 16, with a history of recurrent angina reported an episode of choreic movements 2 years before requiring treatment with haloperidol and prevention of rheumatic fever. The patient interrupted her treatment with occurrence of a relapse a few months later. Brain MRI and transthoracic ultrasound were normal. Preventive treatment with extencilline and haloperidol was restarted without any improvement, hence the treatment with sodium valproate. The patient responded very well after 2 months of treatment, without recurrence at 3 years' follow-up. The treatment of Sydenham's chorea was based on neuroleptics. Recent studies advocate the use of other more effective and better tolerated molecules.

Introduction: Parry-Romberg syndrome is a rare entity of unknown etiology, characterized by hemifacial or hemibody atrophy affecting subcutaneous tissues. Its main clinical manifestations are neurological, ocular and dermatological. Paroxysmal kinesigenic dyskinesia (PKD) is characterized by brief episodes of choreic/dystonic movements triggered by sudden movements and improved by antiepileptic drugs particularly carbamazepine. It can be sporadic or familial with an autosomal dominant inheritance. Little is known about the pathophysiology of PKD, although a channelopathy is thought likely. The association of these two diseases is rare., Case Report: Over three years, a 42-year-old patient progressively developed left hemiatrophy. Since age 14, he presented paroxysmal dystonic disorders of the limbs triggered by sudden movements and controlled by carbamazepine. The diagnosis of PKD associated with Parry-Romberg syndrome was established. Investigations revealed immunologic and diffuse electrophysiological abnormalities (EEG and EMG)., Conclusion: PKD associated with Parry-Romberg syndrome is very rare but does not appear to be fortuitous. It suggests a common mechanism via an underlying channelopathy or dysimmune disorder.

Introduction: Paroxysmal kinesigenic dyskinesia (PKD) is characterized by brief episodes of dystonia and choreoathetosis triggered by sudden voluntary movements. Disease onset is seen in the first or second decade. The attacks typically last less than one minute. Three autosomal dominant PKD loci are identified: EKD1, EKD2 and EKD3. EKD1 has an overlap with the locus of the "Infantile Convulsion and Choreoathetosis (ICCA) syndrome". The favorable natural history, the episodic nature of the symptoms and their sensitivity to anticonvulsant therapy suggest channelopathy as a mechanism of PKD., Patients and Methods: We reviewed the clinical features, the family history, the treatment response, the evolution and the technical investigations in 19 affected individuals., Results: All cases were idiopathic. Ten patients had a positive familial history. Three patients suffered from ICCA syndrome. Some atypical features were seen, such as the association of kinesigenic and nonkinesigenic attacks and the presence of migraine, ataxia, seizures and myoclonus. Acetazolamide responsiveness was seen in two patients., Conclusion: The coexistence of PKD and nonkinesigenic dyskinesia in several patients confirms the earlier described presence of intermediary forms, nonrepresented in the current classification of paroxysmal dyskinesias. Our study results suggest channel dysfunction and basal ganglia involvement in the pathophysiology of PKD.

Introduction: Infection of the central nervous system with human immunodeficiency virus (HIV) can be associated with movement disorders., Case Report: A case of chorea during HIV encephalitis which responded well to antiretroviral therapy is reported. Choreic movements disappeared with a decrease of MRI lesions observed in basal ganglia., Conclusion: The efficacy of anti-retroviral therapy in choreic movements, a rare syndrome with HIV encephalitis, can be underlined.

Myoclonic dystonia is mainly described as a familial entity. Nevertheless it is also a syndrome. In the first part of this review we discuss the diagnostic difficulties of myoclonic dystonia which are mainly explained by the numerous denominations. In a second part, these entities (essential myoclonus, benign hereditary chorea, myoclonic dystonia with dramatic response to alcohol) are described, then grouped into one single disease, namely inherited myoclonic dystonia, To date, only benign hereditary chorea family, mapped to chromosome 14q, is still considered as a separate disease. In a third part, the main causes of myoclonic dystonia syndrome are described, with special focus on inherited myoclonic dystonia or myoclonus-dystonia. Numerous mutations are described on the epsilon-sarcoglycan gene located on chromosome 7q21. The function of epsilon-sarcoglycan is still unknown. The clinical features are predominant alcohol-sensitive myoclonus (neck, arms) with mild and more restrained dystonia (torticollis, writer's cramp). Obsessive-compulsive disorder may be associated with the disease. Promising treatments may be medical (gamma-hydroxybutyric acid) and surgical (deep brain stimulation) although therapeutic abstention may be possible owing to the frequent benign course of the disease.

Two seventeen year-old women, developed acute onset left choreic movements following two months and two weeks use of oral contraceptives. Left hemiparesia appeared a few days later, while involuntary movements discontinued. Cranial CT scan and MRI showed bilateral ischemic lesion in the frontal region for the first case and isolated lesion in the right centrum ovale for the second. Angiography showed nearly complete obstruction of the terminal portion of the internal carotid artery with an outline Moya-Moya network. After discontinuing oral contraceptives, there has been no relapse of neurologic dysfunction for more than three years for the first case and twelve months for the second one. The role of perfusion insufficiency in limb-shaking carotid transient ischemic attack is discussed and the possible relations between oral contraceptives, chorea and angiographic features resembling Moya-Moya disease are evaluated.

Three HIV seropositive patients presented with cerebral toxoplasmosis which was treated by anti-infectious agents. After partial improvement, they developed hemichorea related to striatal infectious lesions. In AIDS patients with cerebral toxoplasmosis, autopsy series have reported a high incidence of basal ganglia abscesses, explaining the occurrence of involuntary movements such as hemichorea.

Motor attacks induced by voluntary movements are infrequent. Paroxysmal kinesigenic choreoathetosis (PKC) is rare and has only recently been individualized (Kertesz, 1967). We report the case of an 8 year-old boy who developed unilateral or bilateral attacks of abnormal, choreoathetotic movements during certain voluntary movements, especially when rising after a rest. The attacks were short (13 to 18 seconds) and frequent. Neurological examination was normal, as were the intelligence quotient, the inter-critical and critical EEG: CT and MRI. The patient's mother had suffered from the same disorder. The attacks disappeared during treatment with phenytoin but reappeared when it was stopped. This case is concordant with data from the literature, with male predominance, age from 6 to 15 years at the onset, shortness of the attacks (less than 1 minute in 80% of the cases) and normality of investigations in almost every patient. A familial factor has been found in 50% of the cases. The frequency of epilepsy in the family is above average. PKC can easily be distinguished from Mount and Reback' syndrome where the attacks are choreoathetotic but longer and unprovoked by movements and where there is also a familial factor. The relationship of PKC with epilepsy is asserted by some authors and denied by others, and in the literature the distinction between movement-induced tonic seizures and PKC is not always clear. Some authors have blamed a disturbance in the maturation of basal ganglia. To sum up, PKC is a very rare condition which is easy to diagnose, has a good prognosis and readily responds to antiepileptic drugs.

A univocal disorder of dopaminergic activity in the nerve structures responsible for extra-pyramidal motility does not take into account the total phenomena seen in psychomotor neurological studies. The hypothesis of two categories of dopaminergic receptors-respectively excitatory (DA) and inhibitory (DAi)--in the striatal and mesolimbic structures concerned, was derived from experimental and clinical anatomical cytological, and pharmacological data. This duality of the receptors could establish a relationship between dopaminergic mediating, mechanism and adrenergic mediation for with the existence of a least two types of distinct receptors, alpha and beta, has been demonstrated. The author discusses the physiopathology of neuromotor disorders wuch as Parkinson's disease, chorea, and various types of dyskinesias, in relation to this dual mediation. By separation of dopamine antagonists and agonists in this new context, it should be possible to have a more selective approach, which would be better adapted, therefore, for the treatment of these affections. The study of the effects of a new medication, tiapride, on dyskinesias provoked by levodopa in parkinsonian patients, is a step forwards in this new method exploration in neurology.

Two cases presenting the main features of the continuous muscle activity syndrome (neuromyotonia) are reported. The clinical particularities between the two patients are under lined. The second case is special because of its acute and reversible evolution and the possible role of gold salts in its etiology. This latter point suggests Morvan's fibrillary chorea. The possible relationships between the two syndrome are discussed.

Denervation, provosed at the turn of the century and abandoned because of inconstant results, has again been taken up in principle, but with a notable change in technique. It is necessary to do a careful examination of the muscles that are to be denervated (of the median and ulnar nerves) and to determine the nature of the spasticity. The incision, longer than in the original technique, allows a better view of the median and ulnar nerves. The nerve branches to each muscle are thus easily identified, and by electric stimulation during the operation one judges how much denervation to subject the muscles to while taking into account the phenomenon of the take-over of the denervated muscles by the remaining nerve fibers. Part of the nerve branches are then sectioned under microscopic control near the muscle, taking care to cauterize the proximal stump. It may be necessary to do associated tenotomies or capsulotomies. This method gives good results if the technique and the contraindications, (athetosis, chorea and cerebral deficits) are respected. It must be emphasized that the results depend on the degree of denervation, which is hardly quantifiable, and is subject to the operator's experience.

A 26 year-old woman presented with a left hemichorea while taking oral contraceptives (a combination of ethinyl-estradiol and norethisterone). There was marked improvement of the choreic movements after discontinuation of the oral contraceptive and complete resolution by 6 weeks. Induction of chorea is a rare complication of oral contraceptives. The possible mechanisms are briefly reviewed.