The rapid correction or over-correction of hyponatremia is believed by many to be the crucial factor in the causation of central pontine myelinolysis (CPM). Over a 17-year period we found CPM in 10 (7%) of the 139 burn patients examined postmortem but in only 10 (0.28%) of the 3,528 patients in the general autopsy population (p less than 0.001). Each of the burn patients with CPM had suffered a prolonged, nonterminal episode of extreme serum hyperosmolality, whereas most burn patients without CPM had not suffered such an episode. The histologic age of the lesions correlated with the duration of time between the hyperosmolar episode and death. Hypernatremia, hyperglycemia, and azotemia, alone or combined, accounted for the hyperosmolality. No single electrolyte or metabolic derangement was essential, as in at least one burn patient with CPM the serum sodium, glucose, or blood urea nitrogen was normal during the hyperosmolar episode. Hyponatremia was not present in any burn patient with CPM. We conclude that severely burned patients, like alcoholics, are especially susceptible to CPM, and that in burn patients with CPM there is a striking association with serum hyperosmolality. The data also suggest that the rapid correction of hyponatremia exerts its effects by causing an osmotic shift and not because of any specific property of the sodium ion.
Twenty-four patients with leukemia or lymphoma refractory to conventional chemotherapy were given a course of systemic, high-dose cytosine arabinoside (3 gm/m2 every 12 hours for twelve doses). Four patients developed cerebellar degeneration during treatment. Ataxia of gait and limb movements, dysarthria, and nystagmus appeared five to seven days after the first dose, worsened over the next two to three days, and then remained stable for two to six days. Incomplete improvement occurred over the following one to two weeks. Postmortem examination disclosed loss of Purkinje cells in the depths of cortical sulci with relative preservation of those at the crests of folia and those in the most posterior inferior portions of the cerebellum. Other patients developed a mild, reversible cerebellar syndrome over the same time course as that of the irreversible disorder. Manifestations ranged from nystagmus alone to dysarthria and unsteadiness of gait without limb ataxia. We conclude that cytosine arabinoside at this dosage causes a cerebellar degeneration with characteristic clinical and pathological features. Among the present patients with refractory hematological malignancies, such degeneration occurred with an incidence of 16.7%, more than twice that reported in previous series.
A retrospective autopsy study of 627 patients with systemic cancer disclosed 153 patients with metastasis to the central nervous system (CNS) and 13 patients with intramedullary spinal cord metastasis (ISM). Thus, the frequency of ISM was 8.5% of cases of metastasis to the CNS and 2.1% of all cases of cancer. Bronchogenic carcinoma accounted for 11 cases of ISM, and breast carcinoma and melanoma for the other two. There were two distinct patterns of spinal cord involvement, indicating spread of tumor to the cord by two different routes. In nine of the 13 ISM patients a metastasis was found deep within the spinal cord, unassociated with leptomeningeal carcinomatosis; this most likely resulted from hematogenous spread of tumor from a pulmonary source. In the other four patients there was focal or multifocal direct extension of leptomeningeal metastatic tumor across the pia into the parenchyma of the cord. Only four of the 13 patients had a clinical myelopathy; in three of these four this was the presenting feature of an occult lung cancer.
Two siblings with Gaucher's disease developed a chronic, slowly progressive neurologic disorder in early adult life. Stimulus-sensitive myoclonus, generalized seizures, supranuclear gaze palsies, and cerebellar ataxia were the main clinical features. Autopsy disclosed perivascular Gaucher cells in the brain, and nerve cell loss and neuronophagia in the brainstem, cerebellum, and spinal cord. From these observations and the literature, there seem to be three clinically distinct neurologic syndromes in Gaucher's disease, depending on the age at which symptoms begin, but the neuropathologic changes are essentially the same in all of them. A modified classification of Gaucher's disease, based on these observations, is proposed.
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