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Biomedical subjects

J M Cler

Publications and source records attributed to J M Cler.

At least 19 recordsLinked to original sources

[Paroxysmal kinesigenic choreoathetosis: autonomic disease or reflex epilepsy?].

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.

Athetosis↗

[Primary reading epilepsy. 11 cases].

Eleven cases of primary reading epilepsy are reported. They confirm Bickford's description. These patients were otherwise normal. A family history of seizures was present in 6 cases. The first seizure occurred between 12 and 25 years. After a variable period of reading all patients developed a tightness and jerking in throat and jaw muscles. In 4 cases, occasionally, speaking could cause jaw or throat jerks. Whilst reading out loud or silently (exclusively in reading aloud in 1 patient) bilateral discharges were recorded in EEG, sometimes on the left hemisphere. The mechanisms are discussed. Seizures in primary reading epilepsy are facilitated by saccadic eye movements, impulse language muscles and mental concentration. The role of higher integrating systems of language is emphasized. Therapy is discussed.

Adolescent↗

[D-penicillamine induced myasthenic syndromes in rheumatoid arthritis. Two cases (author's transl)].

The authors report on 2 personal cases, and review 48 published cases of myasthenia induced by D-penicillamine (D-P) treatment in patients with rheumatoid arthritis. The clinical symptoms were not different from those of myasthenia gravis, and no correlation could be found between the total cumulative dose of D-P and the onset on the myasthenic syndrome. In 71% of the patients the neurological deficiency regressed after D-P was withdrawn, but in some cases anticholinesterase treatment had to be continued and thymectomy was contemplated. The most remarkable biological abnormalities were anti-striational antibodies (found in 58% of the cases) and anti-acetylcholine receptors antibodies (found in 4 out of 7 patients tested). These findings are in favour of a genuine myasthenia and against a myasthenic syndrome due to neuro-muscular blockade. While the mechanisms underlying the emergence of these antibodies remains unknown, their presence throws new light on immunological disorders in rheumatoid arthritis.

Adult↗

[Therapeutic use of tiapride in movement disorders].

Tiapridal was given to twenty-four patients suffering from dyskinesias. Therapeutic efficacity seems to the etiology. Not any fonctitonal improvement was observed in parkinsonism with dyskinesia consecutive to Dopa therapy. On the other hand a good efficacity was found in the treatment of choreas with a quite good tolerance; the functional result will depend of the evolutive state of the disease.

Benzamides↗

[Striato-nigral degeneration. Clinical and anatomic study of a case which responded favorably to L-Dopa].

The authors have reported the case of a 65 year old woman followed for almost 4 years with an akineticrigid Parkinsonian syndrome which responded well to levodopa. Waning of response finally occurred and despite the addition of a peripheral dopa decarboxylase inhibitor the patient died suddenly. Autopsy examination revealed pure nigro-striatal degeneration without any additional pathology. The report deals primarily with purity of the Parkinsonian picture and especially with the conspicuous and prolonged effect of levodopa.

Aged↗

[Tuberculous abscess of the central nervous system (author's transl)].

A case of tuberculous abscess of the cerebellum is reported by the authors. A check-up of concerned literature of ten-past years is drawn up. Diagnosis is seldom raised before surgery in spite of former or evolved tuberculosis. Histology and bacteriology are essential for a supportive therapy. The prognosis is favorable in seventy three per cent of the cases. This pathology must not be forgotten in patients who imigrate from countries of high endemic level of tuberculosis.

Central Nervous System Diseases↗