High-Convexity Tightness Predicts the Shunt Response in Idiopathic Normal Pressure Hydrocephalus.

BACKGROUND AND PURPOSE: Although neuroimaging plays an important role in the diagnosis of idiopathic normal pressure hydrocephalus, its predictive value for response to shunt surgery has not been established. The purpose of the current study was to identify neuroimaging markers that predict the shunt response of idiopathic normal pressure hydrocephalus. MATERIALS AND METHODS: Sixty patients with idiopathic normal pressure hydrocephalus underwent presurgical brain MR imaging and clinical evaluation before and 1 year after shunt surgery. The assessed MR imaging features included the Evans index, high-convexity tightness, Sylvian fissure dilation, callosal angle, focal enlargement of the cortical sulci, bumps in the lateral ventricular roof, and deep white matter and periventricular hyperintensities. The idiopathic normal pressure hydrocephalus grading scale total score was used as a primary clinical outcome measure. We used measures for individual symptoms (ie, the idiopathic normal pressure hydrocephalus grading scale subdomain scores, such as gait, cognitive, and urinary scores), the Timed Up and Go test, and the Mini-Mental State Examination as secondary clinical outcome measures. The relationships between presurgical neuroimaging features and postoperative clinical changes were investigated by using simple linear regression analysis. To identify the set of presurgical MR imaging features that best predict surgical outcomes, we performed multiple linear regression analysis by using a bidirectional stepwise method. RESULTS: Simple linear regression analyses demonstrated that presurgical high-convexity tightness, callosal angle, and Sylvian fissure dilation were significantly associated with the 1-year changes in the clinical symptoms. A multiple linear regression analysis demonstrated that presurgical high-convexity tightness alone predicted the improvement of the clinical symptoms 1 year after surgery. CONCLUSIONS: High-convexity tightness is a neuroimaging feature predictive of shunt response in idiopathic normal pressure hydrocephalus.

Pure word deafness and pure anarthria in a patient with frontotemporal dementia.

A 66-year-old right-handed man developed pure anarthria following pure word deafness. In addition to language disorders, his behavior gradually changed and finally included violence against his wife. Brain magnetic resonance imagings revealed atrophy of the left perisylvian area, which included the inferior half of the precentral gyrus and the upper portion of the superior temporal gyrus, consistent with frontotemporal dementia (FTD). It has been documented as either a disorder of expressive language or as an impaired understanding of word meaning. Unlike with pure anarthria, pure word deafness is not included in the clinical diagnostic current criteria for FTD. However, a large variety of language symptoms can appear in FTD according to the distribution of pathological changes in the frontotemporal cortices. This case suggests that pure word deafness could be a prodomal symptom of FTD.

Adult-onset leukoencephalopathy with vanishing white matter with a missense mutation in EIF2B5.

We report of a woman aged 52 years born to consanguineous parents and seeking treatment for progressive dementia and delusion. Neurologic examination revealed dementia and emotional instability, indifference, and confabulation. There was also mild spasticity of the bilateral lower limbs. MRI revealed diffuse white matter hyperintensity on T2-weighted images accompanied by hypointense areas on fluid-attenuated inversion recovery images. A homozygous missense mutation was identified in EIF2B5.

[Difference between transcortical sensory aphasia following the left frontal lesion and transcortical sensory aphasia following the left posterior lesion].

We assessed the difference between transcortical sensory aphasia (TCSA) following the left frontal lesions (F-TCSA) and TCSA following the left posterior lesions (P-TCSA). All the patients were right-handed and the 7 patients had the lesions in the only frontal lobe and the 10 patients had the lesions only in the left temporo-parieto-occipital regions. We administered pointing tasks, using 90 line drawings representing single nouns. We presented 6 line drawings a pointing board, and we used two kinds of pointing boards: one showed the line drawings each belonging to different categories (random categorized pointing task), the other showed the line drawings each belonging to only either two different categories (two categorized pointing task) and we presented 15 pointing boards each alternatively. The result was that regarding the patients of P-TCSA showed different number of correct answers between the random categorized pointing task and the two categorized pointing task with statistical significance. Regarding the patients of F-TCSA showed no difference between them. The results indicated that disturbance of P-TCSA on the pointing task was the disturbance of semantic process per se. And the disturbance of F-TCSA on the pointing task was that of not only semantic process but also the whole process including comprehending the presented words, searching the line drawings, comparing the line drawings with the presented word and final selection, which demanded persistent multiple memory process consistent with working memory.

[Functional difference between the left supplementary motor area and the left premotor area in a task of confrontation naming and word fluency].

We assessed the faculty of confrontation naming and word fluency of the 11 patients afflicted with frontal lobe infarction or hemorrhage. All the patients were right-handed and manifested transcortical motor aphasia due to cerebrovascular diseases. We carried out the Western Aphasia Battery, and we adopted V-A; the naming task involved confrontation naming of 20 objects, and V-B; the word fluency task involved the naming as many animals as possible in a one minute period. Six patients who have lesions in the left medial frontal lobe performed excellency in the confrontation naming task but exhibited poor word fluency, and 5 patients who have lesions in the left dorso-lateral frontal lobe performed poorly in both tasks. This results suggests that the left dorso-lateral frontal lobe is important in confrontation naming, while the left medial frontal lobe is important in word fluency. Mushiake et al. (1991) showed that the premotor area was involved in visually guided sequential movements, and the supplementary motor area was involved internally determined sequential movements in primates. Regarding language function as analogous to movement, confrontation naming is analogous to visually guided movements and word fluency is analogous to internally determined movements. Thus, our results suggest that the functional difference between the left medial frontal lobe, which includes the supplementary motor area, and the left dorso-lateral frontal lobe, which includes the premotor area, which was demonstrated in primates for movement is also true of language function in humans.

[A case of chronic enteroviral meningitis and hydrocephalus associated with Bruton type agammaglobulinemia].

We report a 10-year-old boy with chronic enteroviral meningitis associated with agammaglobulinemia (CEMA) and hydrocephalus. He was treated with a low-dose intravenous administration (100 mg/kg/4 weeks) of gammaglobulin (gamma-gl) since he was diagnosed as having Bruton type agammaglobulinemia at 1 year of age. At this admission, neurological examination revealed meningeal signs, Babinski sign, frontal signs, urinary incontinence, and mental retardation (IQ = 48) which was considered to be a sequela of the enteroviral encephalitis which had occurred in his first year of life. T 1-weighted MR imaging of the brain following gadolinium administration revealed a marked dilatation of the lateral ventricles and dense enhancement of the meninges. Enterovirus was detected in the cerebrospinal fluid (CSF) using tissue culture. Histological examination of a biopsied leptomeningeal specimen revealed inflammatory thickening, which was a likely cause of the obstruction to the flow of CSF. The hydrocephalus in this patient was treated with external drainage of CSF from the lateral ventricle. The CEMA was brought into remission by means of the intraventricular administration of gamma-gl, at a dose of 125-250 mg/week (total dose: 1.5 g/8 weeks), in addition to the high dose intravenous administration (400 mg/kg/4 weeks) of gamma-gl. Because of the poor prognosis of patients with CEMA, the intraventricular administration of gamma-gl should be initiated immediately following a diagnosis of enteroviral meningitis.

[Posterior cortical atrophy with incomplete Bálint's syndrome].

We report a patient of posterior cortical atrophy (PCA) with progressive memory disturbance and incomplete Bálint's syndrome consisting of optic ataxie (ataxie optique type) and visual inattention without psychic paralysis of fixation of gaze. The patients is a 58-year-old woman who noticed memory disturbance at 53 years old. Neurological deficit at 54 years old was detected only in the domain of memory, and mild diffuse brain atrophy was revealed on MRI. Memory disturbance progressed gradually, and at the age of 58 she was noticed to have visual disorder. Neuropsychological examination revealed severe memory disorder, incomplete Bálint's syndrome, transcortical sensory aphasia, mild ideational apraxia, and severe constructional apraxia. Visual inattention was too severe to evaluate visual acuity and visual field. MRI showed moderate dilatation of bilateral lateral ventricles, especially in their posterior horns, with atrophy of bilateral temporo-parieto-occipital lobes and hippocampus. IMP-SPECT revealed a diffuse decrease of cerebral blood flow in the bilateral temporo-parieto-occipital region, predominantly in the parietal regions. We believe that she is still in the early phase of PCA, and that psychic paralysis of fixation of gaze, visual agnosia will be noted in several years. Our patient represents an example of early stage PCA from neuropsychological and MRI findings.

[Double-blind comparison of L-keflex and cephalexin (Keflex) in dental infections (author's transl)].

In order to evaluate efficacy and safety of L-Keflex (granule form of sustained release cephalexin), a double blind study comparing it with Keflex (capsule of regular cephalexin) was conducted in dental infections. Evaluable cases in adults for efficacy of the drugs were 196 consisting of 97 for L-Keflex and 99 for Keflex. Those in children were 19 (8 for L-Keflex and 11 for Keflex). There were no significant differences in background of the patients and severity of the diseases between both groups (L-Keflex and Keflex groups). The daily doses used in both groups were 1,000 mg in adults and 500 mg in children, respectively. The dose was given in two divided doses for L-Keflex group and in four divided doses for Keflex group. Following are evaluation by the committee members for the study: Adults 1. Clinical response rate at final therapy day was 93.8% in L-Keflex group and 92.9% in Keflex group, showing no significant difference between both groups. 2. No significant difference in severity of subjective and objective symptoms between both groups was observed at each therapy day. 3. Side effects were found in 6.7% of 105 patients receiving L-Keflex and in 5.6% of 107 patients with Keflex, and there was no significant difference between both groups. As the side effects, gastrointestinal symptoms, rash and itching were observed, but no any other side effects were found in both groups. Children 1. As shown in the above, number of the cases enough to evaluate statistically was not obtained, but all of both groups clinically responded to the drugs. 2. As for side effects diarrhea was observed in only one patient of Keflex group consisting of 12 patients. In the patient, however, discontinuation of the drug was not required and the side effect disappeared during the therapy. From the above results, L-Keflex (granule) is judged to have more convenience than Keflex (capsule) in that (1) it can be administered with b.i.d. regimen and (2) it can be easily taken in dental patients such as patients having difficulty in opening mouth of swallowing pain.