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[Possibilities of studying the auditory evoked potentials in patients with various forms of extrapyramidal pathology].

As many as 47 patients with different extrapyramidal hyperkinesias were examined. In addition to the methods commonly accepted in otoneurology, use was made of the method of recording short-wave auditory evoked potentials (SAEP). The parameters of SAEP were correlated to the degree of auditory and vestibular function impairment. The data obtained indicate that the majority of patients with hyperkinesias manifest a well-defined reduction of the amplitude and an increase of the latent period of the late waves of SAEP, which attests to the interest of the cerebral structures.

Acoustic Stimulation↗

[Multiple sclerosis in childhood].

The authors discuss the problems of the age-associated range of multiple sclerosis, characteristics of its clinical manifestations and course in childhood. Provide two cases of multiple sclerosis that had set in 11-year- and 14-year-old children. The first case was marked by a polysymptomatic debut with retrobulbar neuritis of the optical nerves, astereognosis and atetoid hyperkinesis in the hand. The second case was characterized by remitting oculomotor, vestibular, pyramidal disorders associated with facial neuropathy.

Athetosis↗

[An autopsy case of primary cerebral malignant lymphoma initiated with choreoathetosis].

An autopsy case of primary cerebral malignant lymphoma with choreoathetosis as initial and main symptom was reported. A 57-year-old woman showed choreoathetosis in right upper and lower extremities, and mental aberration. Cranial CT scan showed high density areas in bilateral frontal lobes, right caudate nucleus and putamen, right vermis of cerebellum, left corpus callosum, all of which revealed increased high density by enhancement. The diagnosis of malignant lymphoma was confirmed by the findings of CT scan-guided stereotaxic biopsy. Although the consciousness became gradually drowsy, neurological signs and symptoms and cranial CT scan's findings were improved by radiation therapy. Subsequently, she developed paresis of left upper and bilateral lower extremities and died of sepsis and disseminated intravascular coagulation 8 months after the onset. Neuropathological examination revealed macroscopically atrophy and brawnish discoloration in bilateral caudate nuclei and right globus pallidus. Microscopically, there were invasions of tumor cells in the subependymal perivascular space of ventricles and subarachnoidal spaces of cerebellum and brainstem. The bilateral heads of caudate nuclei revealed severe atrophy, neuronal loss and astrocytic proliferation induced by tumor cell invasion into the head of caudate nuclei, of which body and tail were well preserved. The globus pallidus and putamen did not show any abnormalities on left side, but the right globus pallidus was atrophic, and middle part of putamen and globus pallidus showed tissue rarefaction, loss of myelin, and astrocytic proliferation. However, neuronal cells were relatively preserved. In the spinal cord, the tumor cells invaded to the subarachnoidal and perivascular spaces and necrosis of spinal parenchyma were noted from lower cervical to upper thoracic cord.(ABSTRACT TRUNCATED AT 250 WORDS)

Athetosis↗

[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↗

[Chorea mollis, a rare variant of chorea minor].

A 15-year-old boy was admitted at age 10 because of muscle weakness, choreatic involuntary movements, and a profound hypotonia. He was diagnosed as having chorea mollis, a rare variant of Sydenham's chorea. He made a full recovery and received prophylaxis with monthly injections of benzathinebenzyl penicillin for the next 5 years. Six months after discontinuation of the prophylaxis he had a recurrence. The choreatic movements were successfully treated with haloperidol. However, a hypotonia and mild psychiatric symptoms still persist one year later. The clinical course, observed in our patient, is in concordance with the results of recent studies, reporting, that a number of patients with Sydenham's chorea may be left with mild to moderate neurologic and psychiatric sequelae.

Adolescent↗

Validity of messages from quadriplegic persons with cerebral palsy.

Interpreting gestures from retarded, nonvocal subjects is scientifically risky. Investigators must construe the subject's meaning without reference to an external validity measure. A procedure was devised in which message content was provided to nonvocal, severely palsied quadriplegic subjects in advance. Subjects' responses were limited to yes/no gestures. Another investigator elicited the messages without prior knowledge of their content. Results, which indicated reasonably high correspondence between stimulus messages and messages elicited, suggest that such subjects can present the content of their own phenomenal field accurately and that investigators' interpretations need not be considered imaginary.

Adult↗

[Kinesic paroxysmal familial choreoathetosis: presentation of a case].

Report of a case of kinesic paroxysmal choreoathetosis in a family (as far as we know the first case of a family on record in Spanish) which responded very well to treatment with low doses of carbamazepine. The characteristics of this infrequent clinical entity are also discussed.

Athetosis↗

[Abnormal movements of vascular origin].

Three patients with hemichorea and ten with dystonia of vascular origin are reported. Five were secondary to ischemic infarcts, two to lacunar infarcts, three to intraparenchymal hematoma, and in the remaining three the type of lesion could not be determined. The patients with chorea, as opposed to those with dystonia, presented abruptly immediately after the stroke, and had a regressive evolution and good therapeutic response. The type of dyskinesia was not useful to identify the precise localization of the lesion or to determine its nature. In addition, in 5 patients multiple lesions were found and 5 had release of archaic reflexes or cortical atrophy in CT; this shows the importance of the overall functional impairment and focal lesions in the genesis of dyskinesia. There were sensory deficits in 7 patients. In 2 patients lesions were not found in the CT in spite of the presence of previous hemiparesis; this suggests that this technique has limitations to discover focal cerebral lesions in patients with focal or hemicorporal dyskinesia.

Adult↗