RESUMEN

BACKGROUND: Creutzfeldt-Jakob Disease (CJD) is a degenerative disease of the brain, characterized by rapid and irreversible decline, with dementia, ataxia, myoclonus, and other neurological and neurobehavioral disorders associated with rapidly progressive spongiform encephalopathy. The mode of transmission and basic pathomechanism remain unclear. The clinical picture of CJD is highly diverse, producing a number of variants. MATERIAL AND METHODS: The patient to be described is a 68-year-old Polish female, JR, clinically diagnosed with CJD. The article presents the case history in detail, with particular emphasis on neuropsychological testing, which was initiated when the patient was still lucid and capable of cooperation. The first presenting symptom was agraphia, followed by hemianopsia and other vision disorders, culminating in visual hallucinations. As the progress of the disease accelerated there was rapidly progressive dementia, aphasia developing to organic mutism, myoclonus, hyperkinesia, ultimately loss of all verbal contact or voluntary movement. RESULTS: JR's neuropsychological parameters declined in a period of less than 3 months from near normal to levels characteristic of severe dementia. CONCLUSIONS: The clinical picture here presented is consistent with that of the Heidenhain variant of CJD, with spongiform encephalopathy beginning in the right occipital lobe. Several features of the case remain atypical, however, including the absence of the most common genetic mutation and the patient's long survival after onset.

Asunto(s)

RESUMEN

In a mason aged 48 years gradually hardness, rigidity and painful muscle spasms, slowness of movements and awkwardness with progressing difficulties in walking developed. Only after several months of observation the finding of pseudo-myotonia suggested the diagnosis of Hoffmann's syndrome during hypothyroidism. In a number of neurological syndromes and in unclear muscular signs it is useful to consider hypothyroidism in the differential diagnosis.

Asunto(s)

RESUMEN

The aim of this study was to assess the value of EEG examination for the diagnosis of chronic CS2 intoxication in individual cases. 100 electroencephalograms obtained from a sample of 100 workers suspected of CS2 intoxication, treated in clinical department were analysed. The mean age of the group was 51, the mean duration of exposure was 24 years. Chronic CS2 intoxication was diagnosed in 49 persons, with prevalence of psycho-organic syndrome in 23 of them. Out of 100 EEG records, 35 were normal, 48 in normal limits, 12 within the border zone, 5 records were abnormal. In the group of 49 patients with chronic CS2 intoxication 3 records were abnormal, 6 were in the border zone. EEG disorders were found with the similar incidence in groups that differed as far as the clinical picture and the CS2 exposure period. In the authors opinion results of the EEG investigations cannot be considered as a reliable criterion in the diagnosis of chronic CS2 intoxication. The appearance of EEG abnormalities in workers exposed to CS2 should give rise to perform a more rigorous and extended neurological examination.

Asunto(s)

RESUMEN

The results of 373 EEG investigations were analysed. They were obtained in a period of 4 years in 222 workers with occupational exposure to metallic mercury in the synthesis of acetaldehyde and salt electrolysis. Abnormal or borderline abnormal results were found in 13% of them. Results were compared in groups differing in age, duration of occupation, working post and Hg excretion with urine. Significantly greater frequency of increased Hg absorption was found in the group with abnormal records. The authors stressed the following morphological features of EEG in those exposed to Hg: tendency for seizure activity, loss of ability of following of rhythmic flashes, unchanged frequency of background activity despite increasing degree of other abnormalities, absence of low-voltage tracings, persistence of abnormalities in the records after loss of contact with mercury.

Asunto(s)

RESUMEN

The study was undertaken for assessment of the effect of vinyl chloride and is polymers on the nervous system. A group of 200 patients working in the production of VC were studies. The group comprised 155 men and 45 women aged 19-46 years (mean 40). The duration of employment was over 5 years in most workers (85.5%), and 26% worked over 20 years (mean duration 14 years). On the basis of neurological examinations clinical groups were isolated: without neurological changes (58 workers), with symptoms (54), with organic signs (88). The studied groups were not different with respect to age. The shortest duration of employment was in workers without neurological abnormalities. In the complex of symptoms tension headaches, irritability and sleep disturbances prevailed. Organic signs manifested themselves most frequently with pyramidal abnormalities (52), cerebellar (40), sensory trigeminal nerve involvement (24) and pyramidal-cerebellar syndromes (26). In the group of workers with evidence of organic nervous system lesions significantly more frequently a history of acute poisonings, Raynaud's syndrome and hepatic involvement were found. The neurotic symptoms with minor pyramidal-cerebellar signs and sensory trigeminal neuropathy may be related aetiologically to the toxic effect of vinyl chloride.

Asunto(s)

RESUMEN

The authors report their observations on late sequelae of phosphine poisoning. For 18 months after acute poisoning signs of nervous system damage persisted (objective changes, EEG abnormalities).

Asunto(s)

RESUMEN

In a plant producing vinyl chloride by the emulsion method 200 workers who were exposed to vinyl chloride for 1 to 25 yr (mean 14), 58 (i.e. 29%) were free of complaints and nervous disturbances. An astheno-autonomic syndrome was found in 54 (i.e. 27%) and in 88 (i.e. 44%) in combination with positive neurological findings, i.e. pyramidal syndromes (in 52), cerebellar disturbances (in 38), trigeminal neuropathy (in 24) and extrapyramidal symptoms (in 3), in various combinations - pyramidal + cerebellar in 12, trigeminal + pyramidal in 7, trigeminal + cerebellar in 5. Headaches (48%), nervousness (26%), decrease in physical strength (16%), loss of memory (14%), sleeping disturbances and somnolence were the most frequent complaints. Scleroderma-like skin changes were found in ten subjects, but only six of them had any neurological disturbances. Occupational exposure to vinyl chloride was lower in workers without neurological findings. Frequency of the arterial hypertension was the same in both groups, whereas acroparesthesias, Raynaud's syndrome, and increased gamma GTP serum activity were significantly more frequent in workers with neurological disturbances. Sixty-two per cent of the neurologically positive group and only 24% of the negative group reported euphoric or narcotic states after exposure. This probably indicates episodic exposures to high concentrations of vinyl chloride. This difference points to a possibility that neurological disturbances may be related to short exposures to peak concentrations. The neurological injury may be both a direct neurotoxic effect of vinyl chloride and secondary to vascular disorders.

Asunto(s)

Asunto(s)

RESUMEN

On the basis of an analysis of the clinical condition and electromyographic changes in 9 patients treated with Lioresal and 3 controls attention is called to the favourable effect of Lioresal in hemiparesis of cerebral origin. Lioresal was given in daily doses of 50 mg and no unfavourable side effects were observed. Reduction of muscular hypertonia and improvement of general motor activity was obtained.

Asunto(s)