A profound hemispheric hypoperfusion with relatively small infarcts indicates a progressing stroke.

We determined clinical features of patients who had a profound hemispheric hypoperfusion with relatively small, acute cerebral infarcts. One hundred and thirty-five patients with acute cerebral infarction underwent both magnetic resonance imaging (MRI) and cerebral blood flow (CBF) measurement in the acute phase of stroke. Eleven (8.1%) had a profound hemispheric hypoperfusion with relatively small infarcts. In these patients, magnetic resonance angiography or conventional angiography was performed, demonstrating the internal carotid artery (ICA) stenosis or occlusion on the ipsilateral side of the infarcts. MRI and CBF measurement were repeated one month later. All of 11 patients suffered from a mild consciousness disturbance, and showed a progress of neurological symptoms during a few days, indicative of a progressing stroke. Five patients of 11 concomitantly had atherosclerotic lesions in the arteries distal to the circle of Willis. The remaining six patients had only the ICA lesions. The prognosis of the former was poor and the hemispheric hypoperfusion pattern did not improved. On the other hand, the prognosis of the latter was good and the hemispheric hypoperfusion was recovered soon. In conclusion, if the collateral flow through the circle of Willis could compensate the misery perfusion, the prognosis of low-flow infarctions with ipsilateral ICA lesions might be good.

[Disappearance of globus pallidus hyperintensity in a patient with portal-systemic encephalopathy after occlusion of the shunt vessel].

A 67-year-old woman was admitted to our hospital with confusion and asterixis on January 23, 1994. She had had the same symptoms repeatedly. On admission she was disorientated, and had slurred speech and asterixis. Laboratory data showed hyperammonemia (84 micrograms/dl) with a poor ICG hepatic clearance (ICG15min = 32%), although hepatic failure did not exist. Abdominal ultrasonography, CT scan and liver biopsy showed no evidence of cirrhosis. Celiac arteriography revealed a large shunt vessel connecting the portal vein to the left renal vein. A 1.5 T magnetic resonance imaging (MRI) demonstrated a bilateral and symmetrical hyperintensity of the globus pallidus in the T1-weighted images. Portal-systemic encephalopathy recurred repeatedly in spite of the conservative therapy, and there was no evidence of the portal hypertension. Therefore, the operative procedure of ligation of the shunt vessel was done on February 21, 1995. After the operation, blood ammonia level and ICG hepatic clearance were normalized. She became free from encephalopathy. Twenty-seven months after the operation, the hyperintensity of the globus pallidus in the T1-weighted images completely disappeared. There have been several reports describing that the globus pallidus alterations on the T1-weighted images in patients with liver cirrhosis, manganese intoxication and portal-systemic encephalopathy. To our knowledge, this is the first case that the hyperintense signal of the basal ganglia in a patient with portal-systemic encephalopathy disappeared completely after occlusion of the shunt vessel.

[Adult-onset type II citrullinemia in a patient undergoing continuous ambulatory peritoneal dialysis].

A 57-year-old Japanese man was admitted to Toyama Medical & Pharmaceutical University Hospital with delirium and flapping tremor on April 2, 1997. He had been undergoing continuous ambulatory peritoneal dialysis (CAPD) because of diabetic nephropathy since 1993. Blood chemistry showed slightly elevated plasma ammonia level with no evidence of liver injury, and his portal venography revealed no port-systemic shunt. He was diagnosed as having type II citrullinemia because of an elevated citrulline level on amino acid analysis and very low hepatic argininosuccinate synthetase activity obtained from biopsy specimen of liver. In this case, plasma concentrations of ammonia and citrulline were not so high as those in previously reported cases, although the hepatic argininosuccinate synthetase activity was actually less than 10% of the control value. Owing to CAPD, he was conservatively controlled in a relatively good condition. This indicates that CAPD seems to be a useful therapeutic approach for citrullinemia since liver transplantation is still difficult in Japan.

[Neuroleptic malignant syndrome in a patient with polyneuropathy: mechanism of muscle rigidity and elevated serum creatine kinase levels].

A 49-year-old man was admitted with the chief complaints of muscle weakness and gait disturbance. His neurological examination was compatible with peripheral neuropathy, and laboratory tests revealed IgA monoclonal gammopathy, increased protein content in the cerebrospinal fluid (CSF) without pleocytosis, and slow motor and sensory nerve conduction velocity. He was diagnosed as having chronic inflammatory demyelinating polyneuropathy with IgA monoclonal gammopathy of undetermined significance. The patient was treated with steroid, and plasmapheresis. He became so restless that antidepressant and haloperidol were administered. Then, he became unresponsive, and developed high fever, sweating, tachycardia, and tremor. Examination of CSF showed increased 3-methoxy-4-hydroxy-phenylglycol and decreased homovanillic acid. He was diagnosed as having neuroleptic malignant syndrome (NMS). However, his muscle tonus was still flaccid in his lower extremities that had been suffered from chronic polyneuropathy. Interestingly, his serum creatine kinase (CK) content was only slightly elevated. We suppose that the pathophysiological location of NMS might be primarily central, and that muscle rigidity and elevation of serum CK might not occur, if the peripheral nerves were completely impaired.

[A case of multiple sclerosis with relapses during the third trimester of pregnancy].

We report a 31-year-old woman with a relapse of multiple sclerosis during the third trimester of pregnancy. She had been treated palliatively after the relapse until occurrence of a new symptom and new lesions on MRI, and then she had Cesarean section. After surgery, steroid pulse therapy was started and the symptoms and lesions on MRI resulted in improvement. As a strategy to treat patients with multiple sclerosis in their third trimester of pregnancy, it is appropriate to evaluate disease activity using MRI and to start steroid pulse therapy after delivery.

[Creutzfeldt-Jakob disease with a widespread presence of kuru-type plaques after cadaveric dural graft replacement. An autopsy case].

A case of Creutzfeldt-Jakob disease (CJD) is reported in a 48-year-old woman who had received a cadaveric dural graft after a clipping procedure of a cerebral artery aneurysm in September 1985. In November 1994, she noticed unsteady gait and blurred vision at first. She successively developed ataxic gait, dementia and myoclonus, and became mute. Serial CT scans revealed no abnormal findings, and serial EEGs showed diffuse slow activity without periodic discharge. The patient died in March 1996, 17 months after the initial symptoms. A brain autopsy demonstrated extensive spongiform degeneration in the cerebral neocortex, thalamus, striatum, and cerebellum especially in the granular layer, with associated astrocytosis and marked neuronal loss. Immunohistochemically, PrP plaques, so called kuru-type plaques, were extensively distributed throughout the cerebrum and cerebellum. Moreover, some of these plaques resembled "florid" plaques, which were surrounded by a zone of spongiform change. The PrP gene analysis of her blood and brain tissue revealed no mutations with homozygosity, Met/Met, at codon 129. The unusual features of this case, that is the absence of PSD on EEG and the widespread presence of kuru-type plaques including "florid" plaques, may be similar to the features of "new variant" CJD.