伊藤 彰一

中枢神経真菌症は、クリプトコッカス、アスペルギルス、ムコールなどの接合菌、カンジダが原因となることが多い。種々の要因により確定診断が困難であり、疑診例に対する経験的治療が必要となることも多い。免疫抑制患者に発症することが多いことから予後不良なことも多く、各種真菌症の特徴を適切に把握し、エビデンスに基づいた治療戦略が必要となる。本稿では中枢神経真菌症として頻度の高いクリプトコッカス症を中心に、診断や治療に関して最近の知見も交えて述べる。(著者抄録)

Adult-onset Alexander disease (AOAD) has been increasingly recognized since the identification of the glial fibrillary acidic protein gene mutation in 2001. We report on a 56-year-old man who was genetically confirmed as AOAD with the glial fibrillary acidic protein mutation of p.M74T. He developed spastic tetraparesis, sensory disturbances in four limbs, and mild cognitive impairment without apparent dysarthria and dysphagia. The case was characterized by severe atrophy of the medulla oblongata and upper cervical cord with intramedullary signal intensity changes on magnetic resonance imaging. While AOAD is diverse in clinical presentation, the peculiar magnetic resonance imaging findings of marked atrophy of the medulla oblongata and cervical cord are thought to be highly suggestive of the diagnosis of AOAD. © 2012 S. Karger AG, Basel.

Atrophy of the pontine tegmentum and facial colliculus is a characteristic pathological feature of Machado-Joseph disease. We assessed whether this finding can be detected by conventional brain magnetic resonance imaging. A total of 17 patients with genetically confirmed Machado-Joseph disease, 15 disease controls (spinocerebellar ataxia type 6 and dentatorubral-pallidoluysian atrophy), and 17 normal subjects were examined using a 1.5-Tesla magnetic resonance imaging scanner. The widths of the facial colliculus, pontine tegmentum, and pontine base and the area of the fourth ventricle were measured on axial T2-weighted imaging. Pathological examination was performed in 9 Machado-Joseph disease patients. In addition, visual inspection of the facial colliculus was evaluated by receiver operating characteristic analysis. The width of the facial colliculus was significantly smaller in Machado-Joseph disease patients (0.37 ± 0.16 mm; mean ± standard deviation) than in normal subjects (0.73 ± 0.30 mm; P < .01), whereas the width of the pontine tegmentum was smaller in both Machado-Joseph disease (4.85 ± 0.58 mm) and dentatorubral-pallidoluysian atrophy (4.72 ± 0.59) patients than in normal subjects (6.35 ± 0.74 mm; P < .01). Visual evaluation of the facial colliculus showed sufficient area under the receiver operating characteristic curves to differentiate Machado-Joseph disease from dentatorubral-pallidoluysian atrophy (0.78) and spinocerebellar ataxia type 6 (0.87). Pathological evaluation showed significant atrophy of the facial colliculus in all Machado-Joseph disease patients. Atrophy of the facial colliculus is a feasible magnetic resonance imaging finding for diagnosing Machado-Joseph disease, and it is easily found as a flattening of the fourth ventricular floor. © 2012 Movement Disorder Society.

A 58-year-old immunocompetent man gradually developed loss of appetite, cognitive decline, gait disturbances, and personality changes over 4 months. Brain magnetic resonance imaging (MRI) revealed bilateral diffuse leukoencephalopathy without mass formation on admission. His condition progressively deteriorated, and we treated him with intravenous high-dose steroids. His symptoms improved rapidly, but exacerbated when therapy was withdrawn. A brain biopsy was performed, and the diagnosis of primary central nervous system lymphoma (PCNSL) was confirmed. He was successfully treated with high-dose methotrexate therapy. Although it is difficult to diagnose PCNSL without mass formation in the early stages, steroid responsiveness is important and brain biopsy is essential for the correct diagnosis of PCNSL. © 2012 The Japanese Society of Internal Medicine.

Background Urinary dysfunction is common in Parkinson's disease (PD); however, little is known about urinary dysfunction in early and untreated PD patients.Methods Fifty consecutive untreated PD patients (mean age, 66.7; mean disease duration, 23.6 months; and mean Hoehn & Yahr scale, 1.9) were recruited; those with other conditions that might have influenced urinary function were excluded. Patients were evaluated using a urinary questionnaire and urodynamic studies.Results Sixty-four per cent complained of urinary symptoms (storage, 64.0%; voiding, 28.0%). Urodynamic studies showed abnormal findings in the storage phase in 84%, with detrusor overactivity (DO) and increased bladder sensation without DO in 58.0% and 12.0% of patients, respectively. In the voiding phase, detrusor underactivity, impaired urethral relaxation such as detrusor sphincter dyssynergia, and bladder outlet obstruction were present in 50.0%, 8.0% and 16% of patients, respectively. In patients with both storage and voiding phase abnormalities, DO+detrusor underactivity was the most common finding. Few patients experienced urge incontinence and/or quality-of-life impairment owing to urinary dysfunction; none had low-compliance bladder or abnormal anal-sphincter motor unit potential. These urinary symptoms and urodynamic findings were not correlated with gender, disease severity or motor symptom type.Conclusion Urinary dysfunction, manifested primarily as storage disorders with subclinical voiding disorders and normal anal-sphincter electromyography, occurs in early and untreated PD patients. In cases with severe voiding disorder and/or abnormal anal-sphincter electromyography, other diagnoses should be considered.

1)学習成果基盤型教育で順次性のあるカリキュラムを作成するためにHardenらが提唱したラセン型カリキュラムの考え方を参考にした。2)カリキュラムの順次性を医師の育成過程として捉え、Millerの学習ピラミッドを応用して医師へと続くコンピテンスの発達モデルを作成した。3)発達モデルに基づくコンピテンスレベルをユニットの学習目標に反映し、授業とコンピテンスの関係を学習ガイドで学生、教育双方が理解できるように可視化している。(著者抄録)

Objective: Sweating on the palms of the hands and soles of the feet, so-called emotional sweating, is considered to be mediated by the limbic system, including the amygdala and anterior cingulate cortex. To reveal involvement of the limbic system in emotional sweating, we evaluated emotional sweating on the palms in patients with limbic encephalitis. Methods: Sweat and skin vasoconstriction responses to arousal stimuli were recorded on the palms of 7 patients with limbic encephalitis caused by viral infection (n = 3) or immune-mediated encephalitis (n = 4). All patients had amnesia, and magnetic resonance imaging revealed mesial temporal lobe lesions, including those on the amygdala, in 6 of these patients. Results: Sweat responses were absent or markedly reduced in patients with limbic encephalitis compared to normal controls following deep inspiration (p < 0.05), mental arithmetic (p < 0.01), exercise (p < 0.05), and tactile stimulation (p < 0.01). Skin vasoconstriction responses in these patients were also impaired, but the extent of such impairment was mild compared to that of the sweating reductions. Conclusion: Sweating on the palm was significantly impaired in patients with mesial temporal lesions. Sweating on the palm could be a useful index of limbic function. © 2011 Elsevier B.V. All rights reserved.

Objective: Pathological studies showed both pontine transverse (cortico-ponto-cerebellar) and longitudinal (corticospinal) fibers degenerate in MSA. The objective was to investigate the association between the development of cross sign, degenerations of pontine fibers, and the frequency of pyramidal signs in MSA. Methods: Patients with MSA (n = 26) and healthy subjects (n = 27) were enrolled in this study. Whole pontine transverse and longitudinal fibers were individually traced by diffusion tensor tractography. FA was calculated along each entire tractography. Cross sign was graded as: 0, no cross sign; 1, anterior-posterior line only; and 2, complete cross sign. T2-hyperintense MCPs was graded as: 0, no change; 1, slight signal change; and 2, severe signal change. FA of pontine fibers in MSA patients and that in healthy subjects was statistically evaluated by ANOVA with an overall statistical significance level of 0.05. The frequency of pyramidal signs in MSA was compared between each cross and MCP grade. Results: FA of pontine transverse fibers in MSA patients decreased with the development of cross sign. FA of Cross 2 was significantly lower than that of healthy subjects (p = 0.003). As regards pontine longitudinal fibers, FA decreased when cross sign was completed. The frequency of pyramidal signs in MCP 2 and 1 was higher than that in MCP 0. Conclusion: Pontine transverse fibers degenerate as cross sign develop, and degenerations of pontine longitudinal fibers begin, or even accelerate when cross sign becomes apparent. Pyramidal signs are frequently present when T2-hyperintense MCPs are clearly observed. © 2011 Elsevier B.V.

MRI is one of essential tools for neurological diagnosis and useful for assessing contrast differences between lesions and normal tissues and for assessing morphological features of lesions. Here, I introduce published neuroradiological researches conducted in my hospital, especially focusing on features of "signals" and "shapes" of lesions. First, as regards "signals", putaminal T 1-hyperintensity is helpful for diagnosing multiple system atrophy, and it is more useful for discriminating multiple system atrophy from Parkinson disease and progressive supranuclear palsy than putaminal T 2-hyperintensity rim or putaminal T 2-hypointensity. Linear T 2-hyperintensity along the medial margin of the globus pallidus is helpful for diagnosis Machado-Joseph disease. Second, as regards "shapes", posterolateral putaminal linearization is a useful finding indicating putaminal atrophy which is a main feature of multiple system atrophy. Flattened facial colliculus/fourth ventricular floor is helpful for differentiating Machado-Joseph disease from dentatorublopallidoluysian atrophy and SCA6. A contrast enhancement pattern called "cloud-like enhancement" is specific for neuromyelitis optica comparing with multiple sclerosis. In conclusion, all of these MRI features are easy to recognize and useful for diagnosing patients in daily clinical settings. Careful but simple observation of lesions focusing on "signals" or "shapes" may lead to develop new MRI findings which improve our diagnostic abilities.

We describe a 64-year-old woman who developed spinal myoclonus around the left scapula after long thoracic nerve injury by mastectomy. Involuntary muscle twitching was semi-rhythmic, and ultrasonography identified contraction of the serratus anterior, teres major, and rhomboid muscles. FDG-PET imaging revealed markedly increased glucose uptake only in the serratus anterior. Lidocaine injection into this muscle resulted in complete cessation of the involuntary movement, and then she was successfully treated with botulinum toxin type A. These findings raise the possibility that the myoclonus was primarily caused by ectopic firing of the injured long thoracic nerve, then spreading to adjacent muscles possibly via a central mechanism mediated by group Ia afferents. The new imaging tools, such as FDG-PET and ultrasonography, were useful to determine the therapeutic target muscle. © 2010.