Fine structure of inclusion body in the nucleus of Alzheimer glia type II in the brain of hepatocerebral degeneration

Summary Best's carmine positive inclusion bodies in the nuclei of Alzheimer glia type II were observed diffusely in the brain with a special type of hepatocerebral degeneration. These inclusion bodies were examined with an electron microscope. The results showed that a body consisted of -glycogen particles, amylopectin-like substances, and capsules enveloping glycogen particles.It was speculated that the inclusion body is produced and accumulated in the nucleus by the disturbance of glycogen metabolism in the nucleus itself.     

Chronic alcoholism: report of an autopsy case and comparison with pseudoulegyric type of hepatocerebral degeneration

An autopsy case diagnosed as chronic alcoholism was reported. He clinically showed progressive neurological illness. His CNS lesions were composed of disseminated necrotic foci in the cerebral cortices with many Alzheimer type II astrocytes, pachymeningitis hemorrhagica interna, and lesions similar to pellagra and Wernicke encephalopathy. Some clinical and pathological analogous features between chronic alcoholism and pseudoulegyric type of hepatocerebral degeneration were discussed.     

Plasma concentrations of atrial and brain natriuretic peptides in a case with hypertensive encephalopathy

Hemodynamic mechanism for brain edema forrmation in patients with hypertensive encephalopathy is unclear. Potential roles of natriuretic peptides in the pathogenesis of hypertensive encephalopathy are discussed. A 32-year-old man presented with slight left hemiparesis. He was slightly confused, and his blood pressure was extremely high. Cranial plain computerized tomography scans revealed diffuse brain edema mainly in the supratentorial white matter region. Blood examination revealed that plasma concentrations of atrial and brain natriuretic peptides were significantly high. His left hemiparesis disappeared within a day, but he tended to be agitated. His altered mental status, however, resolved with control of blood pressure. Serial magnetic resonance imagings demonstrated that the magnitude of brain edema was attenuated in proportion to decline in plasma concentrations of natriuretic peptides. This case suggests that significant elevation of plasma concentrations of natriuretic peptides may contribute to an acute rise in blood pressure, and that these peptides potentially play an important role in development of brain edema in hypertensive encephalopathy.     

Acquired hepatocerebral degeneration: report of an atypical case

A case of acquired hepatocerebral degeneration secondary to biliary cirrhosis is described. It differs from the conventional type because of the clinical predominance of cerebellar symptomatology and because the principal neuropathological abnormalities were restricted to the pallidum and to the cerebellum.     

Clinical characteristics of acute encephalopathy with acute brain swelling: A peculiar type of acute encephalopathy

Objectives

Acute encephalopathy has been observed with acute brain swelling (ABS) that is characterized by rapid progression to whole-brain swelling. The objective of this study was to describe the clinical characteristics of ABS.

Isolated cortical visual loss with subtle brain MRI abnormalities in a case of hypoxic-ischemic encephalopathy.

A 16-year-old boy who was briefly asystolic and hypotensive after a motor vehicle accident complained of abnormal vision after recovering consciousness. Visual acuity was normal, but visual fields were severely constricted without clear hemianopic features. The ophthalmic examination was otherwise normal. Brain MRI performed 11 days after the accident showed no pertinent abnormalities. At 6 months after the event, brain MRI demonstrated brain volume loss in the primary visual cortex and no other abnormalities. One year later, visual fields remained severely constricted; neurologic examination, including formal neuropsychometric testing, was normal. This case emphasizes the fact that hypoxic-ischemic encephalopathy (HIE) may cause enduring damage limited to primary visual cortex and that the MRI abnormalities may be subtle. These phenomena should be recognized in the management of patients with HIE.     

Diffusion-weighted imaging in a case of wernicke encephalopathy

In a chronic alcoholic patient with progressive confusion, which was consistent with the clinical diagnosis of Wernicke encephalopathy, T2-weighted, FLAIR and diffusion weighted (DWI) MR imaging depicted brain abnormalities located in both medial thalamic nuclei. Apparent Diffusion Coefficient (ADC) measurements in these regions shown unexpected normal values, referring to Wernicke pathological findings and DWI data. DWI may be helpful to diagnose early basal nuclei abnormalities, but may fail to compute ADC values in these locations.     

Characteristic changes on brain CT in a case of Leigh encephalopathy with deficiency of pyruvate dehydrogenase.

A girl aged 23 months of Leigh encephalopathy with pyruvate dehydrogenase complex (PDHC) deficiency was reported. Brain CT scan showed atrophy of the frontal and parietal cortex, low density in the midbrain and putamina, and rounded caudatum. CT change was showed from rounded to atrophic caudatum during a three-month period. The rounded caudatum may be an early CT finding in Leigh encephalopathy.     

Hypertensive brain stem encephalopathy with pontine hemorrhage: A case report

Hypertensive brain stem encephalopathy (HBE) is a rare, under diagnosed subtype of hypertensive encephalopathy (HE) which is usually reversible, but with a potentially fatal outcome if hypertension is not managed promptly. To the best of our knowledge, only one case of HE with brain stem hemorrhage has been reported. We report a case of HBE with pontine hemorrhage in a 36-year-old male patient. The patient developed severe arterial hypertension associated with initial computed tomography showing the left basilar part of pons hemorrhage, fluid-attenuated inversion-recovery showing hyperintense signals in the pons and bilateral periventricular, anterior part of bilateral centrum ovale. The characteristic clinical findings were walking difficulty, right leg weakness, and mild headache with nausea which corresponded to the lesions of MR imagings. The lesions improved gradually with improvements in hypertension, which suggested that edema could be the principal cause of the unusual hyperintensity on magnetic resonance images.     

Acquired hepatocerebral degeneration in a liver transplant recipient

A 47-year-old-man lapsed into coma 12 h after liver transplantation, and remained comatose until death 38 days later. Prior to transplantation he had repeated episodes of hepatic encephalopathy, but no fixed neurological signs. Autopsy revealed typical features of acquired hepatocerebral degeneration with diffuse but patchy pseudolaminar cortical necrosis, variable amount of neuronal loss in the cerebral cortex, basal ganglia and other areas, and proliferation of Alzheimer type II glia. In addition, there was central pontine and extensive extrapontine myelinolysis involving the lateral and medical geniculate bodies, the thalamus, internal capsule, fornix, mamillothalamic tract, white matter bundles in the caudate and pallidum, the oculomotor nuclei and the foliar white matter of the cerebellum. The distinction between myelinolytic lesions and lesions due to hepatocerebral degeneration was not always clear. Although neurological complications and brain lesions are rather common after liver transplantation, there have been no reports of acquired hepatocerebral degeneration in liver transplant recipients. Our data lend support to the idea that a single prolonged comatose episode, due to hepatic dysfunction, may induce permanent parenchymal brain damage.     

Soluble brain proteins in dialysis encephalopathy

Summary Soluble brain proteins were analyzed regionally in a case of dialysis encephalopathy and compared to the findings in patients dying from uncomplicated uremia and acute myocardial infarction. Sixteen individual proteins were quantitated and the results may indicate that dialysis encephalopathy is accompanied by defects in the blood-brain barrier. However, such a postulate does not explain why certain proteins (-trace protein) occur at very low concentrations in dialysis encephalopathy.