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Thanks to the recent advance and development in science and technology extremely small quantities of substances of the cerebrospinal fluid (CSF) can now be measured. However, the measurement of these substances can not be done routinely. While, the classical CSF tests such as CSF pressure, cells, total protein and glucose are still usefull for the daily clinical diagnosis of neurological diseases. Here I report the ordinary CSF findings in acute viral meningitis and encephalitis, though the exceptions are not rare. In Creutzfeldt-Jakob disease the lately reported discovery of the 14-3-3 brain protein in the CSF will make a great impact on the diagnosis and clarification of pathogenesis of the disease.

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Nipradilol is a new type of beta-blocker which possesses nitroglycerin-like vasodilating action in addition to beta-blocking action. We investigated the efficacy and safety of nipradilol for treating tremor in 20 patients with essential tremor (ET group) and 20 patients with Parkinson's disease (PD group). All patients received nipradilol (6 mg per day) for more than 8 weeks. Improvement of tremor appeared within 2 or 4 weeks after the start of nipradilol therapy, and the efficacy rate, defined as "moderately effective" or over, was 42.5% in all 40 patients, while that defined as "slightly effective" or over was 87.5%. The efficacy rate tended to be higher in the ET group compared with the PD group. Mean blood pressure was significantly decreased from the 4th week after the start of treatment and heart rate was significantly reduced from the 2nd week of treatment. Laboratory examination showed no significant changes.

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We cloned and sequenced two new Verotoxin 2 (VT2) variant genes: one from an Escherichia coli strain from a case of bovine diarrhea and the other from an E. coli strain from a patient with diarrhea. The nucleotide and amino acid sequences of these two genes were highly homologous with, but distinct from those of the VT2, VT2vha, VT2vhb, SLT-IIv (VT2vp1) and SLT-IIva (VT2vp2) genes. Their nucleotide sequences were much more closely homologous to that of VT2vh than to that of VT2vp. Search for these two new genes in other Verocytotoxin-producing E. coli strains resulted in the isolation of 2 strains carrying one of the new VT2 variant genes, one strain from Tokyo and the other from Canada.

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A 43-year-old woman had noticed muscular weakness in her arms for four years before her admission. Muscle weakness and atrophy were prominent in the bilateral deltoid muscles, but muscular strength was almost unimpaired in the bilateral forearms and intrinsic muscles. There was no sign of sensory impairment except vibratory sensation. EMG revealed neuropathic NMU. X-P of the cervical spine showed enlargement of the spinal canal diameter, and MRI of the spinal cord revealed a large syrinx. On the basis of metrizamide CT and cranial MRI, a diagnosis of syringomyelia with Chiari malformation (type I) was made. Despite the presence of a large syrinx extending from C 1 to Th 11, the only detectable neurological sign was proximal weakness of the upper extremities simulating myopathy.

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This is the first report to describe the coexistence of two rare diseases, intracranial paramedian hourglass-shaped dermoid and steatocystoma multiplex. A 46-year-old female with a history of steatocystoma multiplex, bradydactylies and kyphosis showed oscillopsia, ataxia and hemifacial spasm. MRI findings suggested a giant dermoid cyst extending from the left middle temporal fossa to the cerebellopontine angle, and this was confirmed surgically. We propose the involvement of some genetic factor or pathological process common to both disorders, steatocystoma multiplex and dermoid. From the clinical point of view, patients with cystic skin lesion should also be checked for intracranial lesion.

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Because a highly sensitive method is required to quantify low concentrations of immunoglobulin (Ig) classes in cerebrospinal fluid (CSF), there have been a few papers reporting normal values of CSF IgG, IgA and IgM determined in the same samples. Enzyme immunoassay (EIA) is most frequently used, but has such drawbacks as susceptibility of enzyme to inhibition and denaturation and the requirement for additional incubation with a substrate. Therefore, solid-phase immunofluorometric assay was evaluated for quantification of CSF IgG, IgA and IgM in the nanogram range. We found this to be rapid and reproducible. The mean (SD) values of normal CSF samples obtained from 22 subjects with tension headache were 23.9 (7.6) micrograms/ml for IgG, 2.00 (0.90) microgram/ml for IgA and 197 (87) ng/ml for IgM. The normal mean (SD) values of indexes were 0.51 (0.10) for IgG, 0.25 (0.05) for IgA and 0.044 (0.017) for IgM. These values agreed quite well with those determined by EIA. The values of CSF albumin correlated significantly with those of CSF IgG or IgA, but did not with those of CSF IgM. Levels of each of the three Ig classes in CSF and serum were significantly correlated. When CSF/serum ratio was introduced, a significant correlation between the albumin ratio and each Ig ratio was found. These results suggest that the Ig content of normal CSF may depend upon that of serum and upon the characteristics of the Ig molecule.

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Calcium hopantenate (HOPA) has been widely used as an activator of cerebral metabolism in Japan. However, several cases of acute encephalopathy during HOPA administration were recently reported, which were characterized by marked metabolic acidosis and hypoglycemia. The encephalopathy in these patients was named Reye-like syndrome because of the similarity to Reye's syndrome in children. The purposes of this presentation are to report on 5 patients with acute encephalopathy developing during HOPA administration, to summarize their symptoms and clinical courses, and to discuss the pathogenesis of metabolic acidosis and hypoglycemia. Initial characteristics of the clinical course in all patients were loss of appetite, nausea and vomiting, followed by unconsciousness. Laboratory examinations revealed marked metabolic acidosis, severe hypoglycemia, hyperlactacidemia, leukocytosis, ketonuria, and increased Ht and BUN. A few days after development of the initial symptoms, mild renal and liver dysfunction, and elevation of serum amylase were observed in all patients. Hyperlactacidemia was present in 4 in the initial period. Blood concentration of HOPA was 2.131 micrograms/ml in patient 1 (8-10 hours after final administration), and 10.7 micrograms/ml in patient 5 (24 hours after final administration). These values are extremely high, because usually HOPA concentration is almost negligible 7 hours after the drug is taken. As the pathogenesis of acute encephalopathy due to HOPA administration, the failure of fatty acid beta-oxidation has been proposed by some investigators. However, the serum concentrations of CoA, pantothenic acid and carnitine during the initial stage were not reduced in our patients. Furthermore, it is very difficult to explain the severe hypoglycemia in terms of the beta-oxidation theory.(ABSTRACT TRUNCATED AT 250 WORDS)

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With canine disease models for aortic dissection, we performed comparative evaluations of several surgical procedures for the management of this dissection. From these experimental procedures, we developed a new operative technique, the Ivalon sponge occlusion method, designed to promote thrombus formation and to mesh (organize) effectively with the tissue ingrowth of the peripheral dilated, dissected aortic lumen. The details of the technique are described. It has been used successfully in 12 patients with DeBakey type I and type III aortic dissection. Based on our experimental and clinical evaluations, although the results are preliminary, we believe this operative technique is a simple, effective approach for the management of extended aortic dissection.

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Two autopsy cases with ruptured dissecting aneurysm of the intracranial portion of the vertebral artery are reported. Both patients developed subarachnoid hemorrhage and died within 9 days after onset. Autopsy disclosed dissecting aneurysms symmetrically on the bilateral vertebral arteries but there was no evidence of underlying vasculopathy in either patient. The etiology of the dissecting aneurysm of the intracranial vertebral artery is obscure, but the repeated minor trauma associated with head movement is considered as an important factor.

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Herpes simplex virus (HSV) antibody titers and IgG, IgM and IgA concentrations in the cerebrospinal fluid were serially measured in a patient with HSV encephalitis during a follow-up period of 32 months. HSV antibody titers, all classes of immunoglobulins and Ig% showed significant elevation during the course of illness, though IgM% and IgA% gradually declined after the acute phase. Autopsied brain tissue failed to yield a virus isolate, but conspicuous perivascular lymphocyte infiltrate, which is compatible with HSV encephalitis, was seen. These observations suggest the occurrence of HSV persistence and persistent antigen stimulation in the central nervous system, analogous to the well-recognized latent condition within the trigeminal ganglion.

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