A retrospective clinical study of 22 heavy alcohol drinkers is reported in which postmortem study showed diffuse chromatolysis of neurons identical to that found in neurological pellagra, associated in 13 cases with Marchiafava-Bignami disease and/or Wernicke-Korsakoff disease. The clinical features included confusion and/or clouding of consciousness, marked oppositional hypertonus ('gegenhalten') and myoclonus. Because of the frequent coexistence of other alcoholic encephalopathies in the same patient, alcoholic pellagra was often unrecognized. Fifteen patients received thiamine and pyridoxine therapy without niacin. It appeared to aggravate the neurological state or to trigger the development of alcoholic pellagra encephalopathy in 9 cases. The relationship between pellagra occurring during thiamine and pyridoxine therapy and 'nicotinic acid deficiency' is discussed. Multiple vitamin therapy should be given in the treatment of undiagnosed encephalopathies in alcoholic patients.

Of the 718 patients investigated for intractable epilepsy by stereoelectrocencephalographic (SEEG) exploration, 30 (4%) manifested gustatory hallucinations as part of their seizures. In 20 patients, it was possible to make some electrophysiological, clinical and anatomical correlates. Gustatory hallucinations occurred as one manifestation of parietal, temporal or temporoparietal seizures. A brief isolated gustatory hallucination was induced mainly by electrical stimulation of the parietal or rolandic opercula in patients with gustatory seizures, in 1 epileptic patient with parietotemporal epilepsy who had never experienced gustatory hallucinations and in another with temporal lobe epilepsy with no history of gustatory manifestations. The electrically-induced seizures, which included a gustatory hallucination as one of the ictal events, were obtained mainly by stimulation of the hippocampus and amygdala. The associated ictal events of a seizure with gustatory manifestations differed depending upon the origin of the seizure. During parietal seizures, they consisted of staring reactions, clonic contractions of the face, deviation of the eyes and salivation. During temporal lobe seizures, the associated events included mainly oral movements, autonomic disturbances, purposeless movements and epigastric or other abdominal symptoms. Seizures affecting both the infra- and suprasylvian regions were characterized by symptoms of both categories listed above. Emotional disturbances were observed mainly when there was an involvement of the cingulate gyrus. When care was taken to avoid methodological errors in the interpretation of the clinical signs occurring after electrical stimulation, it became clear that gustatory hallucinations in man were related to the disorganization of the parietal and/or rolandic operculum. electrically-induced temporal lobe seizures which included gustatory hallucinations as an ictal event probably spread to the opercular region by a functional reorganization of the connections within these epileptogenic areas.

Temperature-controlled radiofrequency ablation was effective in selected patients with mild to moderate obstructive sleep apnea syndrome. A full-night polysomnography is required after completion of treatment to rule out residual disease.

No abstract

We performed a validation study of the diagnostic mode of the Autoset system (ResMed, Australia) on a group of 44 snorers (10 women). We compared the result of the Autoset's automatic analysis of nasal airflow (using nasal prongs) to those of an in-laboratory polysomnographic study with a Fleisch facemask pneumotachograph. For the first 29 patients, the Autoset software was set to recognize only apneas; for the remaining 15, the software was modified to recognize both apneas and hypopneas. Relative to polysomnography, the Autoset overestimated the number of apneas. Oral breathing or displacement of the nasal prongs partially explained these differences. A significant correlation was found between the apnea indices (AI) assessed by the two methods (r = 0.98). For an AI of 20/hour the Autoset was 100% sensitive and 88% specific. The Autoset significantly underestimated the number of hypopneas compared to the polysomnograph with pneumotachograph (62.9 +/- 4.7 vs. 85.5 +/- 73.1, P = 0.04), although for an apnea-hypopnea index of 20, Autoset was 100% sensitive and 88% specific. The lack of linearity of Autoset's volume evaluation at low volumes could explain most of the differences. Our results indicate that the Autoset system, in its diagnostic mode, is a useful tool for identifying patients with significant obstructive sleep apnea syndrome. The system is less useful in patients with mild to moderate sleep disordered breathing, where it may give erroneous results.

A fifth case of oculomasticatory myorhythmia associated with cerebral Whipple's disease is reported. This peculiar abnormal movement has never been described in association with cerebral dysfunction other than Whipple's disease. The present case exhibited rhythmic convergence of the eyes and synchronous (1-2 Hz) contractions of the masticatory muscles and of the proximal and distal skeletal muscles. These abnormal movements occurred 13 years after the beginning of the disease. They were persistent and unchanged until the death of the patient 3 months later. No treatment was effective to suppress the involuntary movements (clonazepam, baclofen, antibiotics). Associated neurological signs included global supranuclear ophthalmoplegia, facial weakness, bilateral ptosis, absent gag reflex, and intellectual deterioration.

Sleep disorders are important manifestations of neurodegenerativediseases and sometimes are clinically evident well before the onset of other neurological manifestations. This review addresses theneuroanatomical basis and the mechanisms of sleep regulation in humans in relation to the neuropathology of entities associated with sleep disturbances in selected diseases, including Alzheimer disease, progressive supranuclear palsy, Lewy body disorders, multiple-system atrophy, and fatal familial insomnia. This includes abnormalities of circadian rhythm, insomnia, narcolepsy, rapid eye movements sleep behavior disorders, and excessive daytime sleepiness.

In 22 patients with alcoholic encephalopathies, chromatolysis similar to that reported in endemic pellagra was found on postmortem examination. No gross macroscopic changes were seen in affected areas and only neurons were involved. The changes consisted of central chromatolysis, seen predominantly in the brainstem, especially in the pontine nuclei, where they were constant, and in the cerebellar dentate nuclei. Nuclei of cranial nerves (mainly the third, sixth, seventh and eighty), the reticular nuclei, arcuate nuclei and posterior horn cells, were also markedly affected. Changes were sometimes seen in the cerebral cortex, the interpeduncular nuclei, the central mesencephalic grey matter, the colliculi, the tenth and twelfth cranial nerve and perihypoglossal nuclei, the gracile and cuneate nuclei and anterior horn cells. This distribution was different from that reported in endemic and 'endogenous' pellagra or in isoniazid-induced pellagra encephalopathy. Central chromatolysis was the only pertinent finding of the CNS examination in 9 cases. In 8 cases, chromatolysis was associated with Marchiafava-Bignami encephalopathy, in 4 cases with Wernicke-Korsakoff encephalopathy, and in 1 other case with both. Mild degeneration of spinal cord tracts was seen in 3 cases. The chromatolysis of alcoholic pellagra did not appear to be a retrograde change related to axonal degeneration. Systemic examination showed liver changes in 15/16 cases. Treatment of these cases had not included niacin. No differences were found between cases given thiamine and pyridoxine and those which had not. Microscopic examination of the pons is essential in alcoholic encephalopathies.