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Four patients with progressive supranuclear palsy presented with various psychiatric symptoms. Periodic stupor-like states due to fluctuations of consciousness were observed in all four cases. Magnetic resonance imaging showed severe dilatation of the third ventricle suggesting atrophy of the thalami. Neuropathological examination in one of the cases confirmed severe fibrillary gliosis in the intralaminar thalamic nucleus. So far, few studies have focused on periodic stupor-like states observed in progressive supranuclear palsy patients. From our cases and from the literature, we postulate that the lesions in the intralaminar thalamic nuclei may have close relevance to the stupor-like states seen in progressive supranuclear palsy patients.

Immunocytochemical staining for glial fibrillary acidic protein (GFAP) allows more specific identification of astrocytes and their processes than classical histochemical techniques and has therefore recently been used by some investigators to quantify gliosis. However, although the immunocytochemical method is superior for delineation of reactive astrocytes, the examples presented here and previous work by others demonstrate that chronic fibrillary gliosis may be best detected by Holzer's method and not by GFAP immunocytochemistry. The authors' studies indicate that if, as in a recent study of gliosis in schizophrenia, computer-assisted densitometry is to be used to measure gliosis, the immunoperoxidase method may not be a sensitive technique to demonstrate glial changes in human postmortem material.

109 brains of children in the age from 16 years down to newborns have been examinated. It was found that in the age between the 2nd month and 5th year there was accumulation of fibrillary gliosis in the brain stem. This cannot be understood entirely as the result of a disease since they were present also in cases of acute death. Therefore, it is concluded that fibrillary gliosis is representative for a physiological intermediate state, and can be increased by general diseases. We have not found any connection between this phenomenon and other formative processes in the development.

The examination of the central nervous system (CNS) of an 11-month-old citrullinaemic patient revealed an ulegyria with a dense fibrillary gliosis and large amounts of lipid phagocytes. Alzheimer type 2 glial cells were present in intact cortical areas. While such cells were related to the insufficiently controlled hyperammonaemia, the ulegyria resulted from impaired oxygenation or impaired blood circulation, possibly influenced by the hyperammonaemia and citrullinaemia.

Summary Histological changes are described in the brain of a patient in whom a hemicerebellactomy had been performed 14 years before death. There is cell and fibre loss, partial or total, in nuclei known to have direct connections with the cerebellum. In some nuclei the sequel to cell loss is marked fibrillary gliosis. In other nuclei the cells have disappeared without trace. The degeneration of these tracts with a direct connection with the cerebellum has also led to degeneration of other tracts in synaptic relationship with them. This transneuronal degeneration has occurred in the corticopontine tracts and in the central tegmental tract, in the former definitely, and in the latter probably, retrogradely. It is probable that transneuronal degeneration has also occurred in other tracts. The extent of the degeneration and, in particular, the occurrence of overt transneuronal degeneration, suggest that the examination of material after a very long term of survival might be of value in experimental work concerned with mapping neuroanatomical networks.

In Rida there is a symmetrical affection of a certain number of brain stem nuclei many of which are related to the cerebellar structures. This consists of neuronal vacuolation with varying degrees of fibrillary gliosis and very slight cell loss.

A 41 year old man had recurrent hypothermia with loss of consciousness which eventually became permanent. There was agenesis of the corpus callosum, with no evidence of dysfunction of the hypothalamo pituitary axis. Autopsy confirmed the agenesis, and showed intense fibrillary gliosis restricted to the arcuate nucleus and premammillary area, which may account for the thermoregulatory disturbance. The association of these hypothalamic lesions with congenital absence of the corpus callosum would seem a mere coincidence were it not for the striking clinical similarities between this case and others previously published. It is suggested that agenesis of the corpus callosum should be looked for in cases of unexplained spontaneous hypothermia.

Summary Th is report presents findings from an unusual case of Pick's disease that was characterized by existence of choreo-athetoid movements and frequent epileptic seizures in the clinical course, and, neuropathologi-cally, by the absence of “wedge-shaped” localized atrophy and fibrillary gliosis in the affected region in spite of thirteen years' clinical course. These findings are compared to those of other case of unclassifiable presenile dementia.

A unique episodic hypothermia, though apparently extremely rare, is currently attracting considerable attention because of its distinctive clinical and pathological features. Also known as recurrent, relapsing, spontaneous, and periodic, this hypothermia usually begins in childhood or adolescence. Characteristically, a sudden bout of profuse sweating and vasodilatation ushers in a precipitous drop of temperature that may reach a nadir of 30 C or less. The episode, which can last anywhere between one half to two hours, may recur many times in a day, with or without prolonged remissions of weeks', months', or years' duration. A curious absence of the corpus callosum was demonstrated roentgenologically in three patients 1,2 and confirmed at autopsy in a fourth 3 —the latter also manifesting heavy fibrillary gliosis with neuronal loss in the premammillary region and in the arcuate nuclei of the hypothalamus. The exact mechanism of this curious hypothermia is poorly understood. It is tempting