[Proton MR spectroscopy studies of the brain in ALS patients]. / Badanie metoda spektroskopii rezonansu magnetycznego u chorych na stwardnienie boczne zanikowe.

Nuclear magnetic resonance spectroscopy (MRS) has an ability to measure brain metabolites noninvasively in vivo. The content of N-acetyl-aspartate (NAA) is used as a biochemical marker of neuronal integrity and viability. In amyotrophic lateral sclerosis (ALS) patients the degeneration and neuronal loss of motor cortex was reported. The presence of these changes can lead to the decrease of NAA. The aim of this study was to evaluate the neurochemical status of motor cortex (by using 1H-MRS) in 11 patients with clinically definite or probable forms of ALS (according to the El Escorial criteria). These data were compared with MRS results from 4 healthy controls. The mean NAA/(Cr + PCr) values were decreased by 19% (p < 0.05) when compared with controls. In 4 ALS patients the NAA/(Cr + PCr) ratio was decreased by 31% (p < 0.05) and these patients had rapidly progressing disease. In other 7 ALS patients, we found that NAA/(Cr + PCr) was decreased by 10% (p < 0.05) and they showed less advanced neurological symptoms. 1H-MRS of the motor cortex can be a new diagnostic tool in ALS and it might help to monitor the progress of the disease.

[Corticospinal tract assessment in ALS: transcranial magnetic stimulation]. / Ocena drogi korowo-rdzeniowej w SLA: badanie metoda przezczaszkowej stymulacji magnetycznej.

Results obtained during transcranial magnetic stimulation (TMS) in 79 patients with ALS were compared to those obtained in 10 healthy volunteers. M and F responses evoked by peripheral stimulation of the ulnar and peroneal nerves and then motor evoked potentials (MEP) induced by TMS were recorded in the abductor digiti quinti (AV) and tibial anterior (TA) muscles bilaterally. Central motor conduction time (CMCT) was calculated using MEP and F response latencies. The MEP/M x 100% amplitude ratios were calculated. Mean CMCT was significantly (p < 0.0001) prolonged in patients with ALS (9.6 +/- 4.1 ms for AV and 20.4 +/- 9.6 ms for TA) and MEP/M x 100% amplitude ratios were higher (45 +/- 75% for AV-p < 0.001 and 27 +/- 32.7% for TA p = 0.2). In a sub-group of patients with predominant upper motor neuron (UMN) involvement, the CMCT was significantly prolonged (14.2 +/- 5.8 ms for AV and 28.5 +/- 9.1 ms for TA), but MEP/M x 100% amplitude ratio was 24.1 +/- 37.6% for AV and 11.4 +/- 12.9% TA. These results confirm usefulness of combined analysis of CMCT and MEP/M x 100% amplitude ratio as the method of assessment of pyramidal tract function in ALS.

[Analysis of the course of amyotrophic lateral sclerosis based on data of the Neurologic Clinic at the Medical Academy in Warsaw 1955-1979]. / Analiza przebiegu stwardnienia zanikowego bocznego (SLA) na podstawie materialu Kliniki Neurologicznej AM w Warszawie z lat 1955-1979.

The natural history of ALS was analysed in a group of 132 cases. Three clinical types were isolated: typical (78% of cases), bulbar (14%) and polyneuropathic (8%). The typical and polyneuropathic types were more frequent in males, while females prevailed in the bulbar type. In most cases the onset was in the fifth decade of life and death occurred within three years. In 11% of cases the disease extended over many years despite presence of bulbar involvement. No familial occurrence of the disease was observed, although this has been reported in the literature. No cases were noted of the ALS-parkinsonism-dementia syndrome.

[A case of hypersomnia resembling Kleine-Levin syndrome]. / Przypadek hipersomni przypominajacy zespól Kleine-Levina.

A case report is presented of episodic hypersomnia with bouts of several days duration. The authors discuss various clinical syndromes with sleep disorders with particular consideration of the Kleine-Levin syndrome.