Diagnostic Value of Brain Calcifications in Adult-Onset Leukoencephalopathy with Axonal Spheroids and Pigmented Glia.

Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia is a rare neurodegenerative disease resulting from mutations in the colony stimulating factor 1 receptor gene. Accurate diagnosis can be difficult because the associated clinical and MR imaging findings are nonspecific. We present 9 cases with intracranial calcifications distributed in 2 brain regions: the frontal white matter adjacent to the anterior horns of the lateral ventricles and the parietal subcortical white matter. Thin-section (1-mm) CT scans are particularly helpful in detection due to the small size of the calcifications. These calcifications had a symmetric "stepping stone appearance" in the frontal pericallosal regions, which was clearly visible on reconstructed sagittal CT images. Intrafamilial variability was seen in 2 of the families, and calcifications were seen at birth in a single individual. These characteristic calcification patterns may assist in making a correct diagnosis and may contribute to understanding of the pathogenesis of leukoencephalopathy.

Power spectral analysis of auditory brainstem responses and MRI findings in patients with spinocerebellar degeneration.

Power spectrum analysis of auditory brainstem responses (ABR) and magnetic resonance imaging (MRI) findings were obtained from 14 patients, 9 with olivopontocerebellar atrophy (OPCA; Dejerine-Thomas type) and 5 with Holmes-type cerebello-olivary degeneration, and compared to data from normal controls. The power spectrum was divided into three components according to frequency: up to 400 Hz (slow component); from 400 to 800 Hz (middle component); and 800 to 3,000 Hz (fast component). In patients with OPCA, the slow component appeared significantly less often than in both groups, normal controls (p < 0.0005) and patients with Holmes-type degeneration (p < 0.005), and the middle component appeared significantly more often than in both groups (p < 0.0005). There was a significant positive correlation between the proportion of the slow component and midbrain diameter (p = 0.04) and a significant negative correlation between the proportion of the fast component and midbrain diameter (p = 0.02). We conclude that the power spectrum differs between OPCA and Holmes-type degeneration, and that the proportion of each component of the power spectrum is related to midbrain volume.

[Serotype determination of enteroviruses that cause hand-foot-mouth disease; identification of enterovirus 71 and coxsackievirus A16 from clinical specimens by using specific probe].

Coxsackievirus A16 (CA16) and enterovirus 71 (EV71) are known to be major causative agents of hand-foot-and-mouth disease prevalent in summer in Japan. Discrimination and identification of these viruses were often hampered by a nonneutralizable or nontypable virus. Therefore, a Southern blot hybridization that utilizes mixed probes specific to serotype was developed. Firstly, an approximately 650 bases spanning 5'-noncoding region to one third of VP2 including entire VP4 was amplified with a set of primers containing enterovirus common sequences and a genomic RNA as template. Secondary, the nucleotide sequences were determined using seven CA16 and eighteen EV71 strains including the standard strains, and the deduced amino acid sequences of VP4 were searched to find residues which are conserved in the same serotypes but diverged among different serotypes. Candidate positions for the mixed probes were defined at the carboxyl terminus of VP4. Thirdly, Southern blot analyses were carried out using thirty-nine enterovirus standard strains, seven CA16 isolates and sixty-six EV71 isolates previously identified by the neutralization test. The results revealed that each mixed probe exclusively bound to the homologous DNAs but not to the heterologous ones. In an attempt to determine serotypes without virus isolation, clinical specimens from hand-foot-and-mouth disease were examined. Of 78 throat swabs and 15 vesicular fluids, 71 (91.0%) and 13 (86.7%) specimens were clearly identified, indicating that the method described here offer advantages over the traditional neutralization assay: It is rapid, specific and less labor-consuming.

[Investigation for VP4 region of coxsackie virus A16 RNA sequence from hand-foot-mouth disease patients at eastern district of Shizuoka prefecture in 1995].

In 1995 an investigation was made for VP4 regions of coxsackie virus A16 (CA16) RNA sequence from hand-foot-mouth disease patients in eastern district of Shizuoka Prefecture. Subjects were seven patients who were diagnosed as hand-foot-mouth disease due to CA16 at the Ohashi Pediatric Clinic in Susono City. Throat swabs of patients were extracted to RNA. Extracted RNA were assayed by reverse transcription polymerase chain reaction that primers corresponded to VP4 resion of enteroviruses. PCR products were marked by dye-deoxy terminator methods and assayed by direct sequence methods. RNA sequences were classified into two types. Type 1 were three cases, and type 2 were four. The homology was 90.8% between type 1 and type 2. All cases of sixty-nine amino acids were the same as prototype strain. We concluded that the two type strains of CA16 were prevalented in eastern district of Shizuoka Prefecture in 1995. It was at the same time and was widely noted in the eastern district.

Relationship between cognitive impairments, event-related potentials, and motor disability scores in patients with Parkinson's disease: 2-year follow-up study.

We studied event-related potentials and the cognitive state for 2 years in 29 patients with Parkinson's disease (PD). Of those patients, 11 were at stage II and 18 were at stage III at initial assessment, as measured on the Hoehn and Yahr scale. The peak latency of P300 in patients at stage III was significantly prolonged, as compared with that in age-matched normal controls or PD patients at stage II. There was no significant change in P300 latency among patients whose motor ability remained unchanged at stage II or stage III during follow-up period. The mean P300 latency prolonged significantly in patients whose motor ability worsened from stage III to stage IV. The cognitive state in the patients with PD was characterized by impairment in the categories of orientation, recall and constructional ability. The degree of impairment of these items increased as the motor disability increased. These results suggest that cognitive dysfunctions and abnormality of P300 latency increased as the motor disabilities progressed.

[A case of neonatal herpes infection which proved to be a mixed infection of herpes simplex virus type 1 and type 2].

We encountered a case which proved to be a mixed infection of herpes simplex virus (HSV) type 1 and type 2 in retrospective terms by in situ hybridization (ISH) and polymerase chain reaction (PCR). The case was a male. The gestational age was 39 weeks and 2 days. The birth body weight was 3024 g. A fever developed from the age of 6 days and he was admitted to the neonatal intensive care unit at the age of eight days. AST was 1042 IU/L, and ALT 206 IU/L. In spite of treatment, the patient died at the age of 12 days. Using paraffin embedded tissues, we performed the ISH and PCR on the cerebrum, lungs, liver, spleen, bone marrow, adrenal gland, and kidneys. With the ISH, the lungs, liver, spleen and adrenal gland were both HSV type 1 and type 2. With the PCR, only the liver was positive for type 1, and the lungs, liver, spleen, and adrenal gland were positive for type 2. In the ISH, a probe showing a cross reaction between type 1 and type 2 was used for type 1 probe this time. But a type 2 probe and PCR did not show a cross reaction. We concluded that this case confirmed the presence of mixed infection (HSV type 1 and type 2) in neonatal HSV infection.

Auditory brain-stem responses and MRI findings in patients with olivo-ponto-cerebellar (Dejerine-Thomas)-type and cerebello-olivary (Holmes)-type spino-cerebellar degeneration.

Auditory brain-stem responses (ABRs) and magnetic resonance imaging (MRI) findings were investigated in 10 patients with olivo-ponto-cerebellar atrophy (OPCA: Dejerine-Thomas type) and in 5 patients with Holmes-type cerebello-olivary degeneration. In patients with OPCA, (1) the III-V interpeak latency (IPL) was shortened significantly (P < 0.05) as compared with normal subjects when the decrease in longitudinal distance of the pons was within 20% of that of normal controls, (2) the I-III IPL was elongated (P < 0.05), the III-V IPL was shortened (P < 0.01), and the amplitude of the V wave was reduced (P < 0.05) when the decrease in longitudinal distance of the pons was more than 20% of that of normal controls. Patients with Holmes-type degeneration showed no significant difference in any component of ABRs or in the size of any part of the brain-stem, as compared with normal subjects. We conclude that abnormalities in the I-III IPL or the III-V IPL in patients with OPCA were produced by changes in the volume conduction in the pons and midbrain. This study also suggests that differences of IPL appear between OPCA and Holmes-type degeneration.

[Subjective symptoms and blood findings in painters exposed to organic solvents].

Subjective symptoms and blood findings were studied in 16 construction workers and 30 vehicle repairmen using organic solvent and compared to 30 workers who were not exposed to organic solvents. A statistically significant increase was observed in the prevalence of subjective symptoms, serum phospholipid, lipoprotein fraction and IgE in construction workers and vehicle repairmen. A statistically significant decrease in neutrophils function was observed in construction workers.