伊藤 彰一

MRI findings of sarcoidosis are usually intraparenchymal granuloma with leptomeningeal lesions. This appearance is dependent upon leptomeningeal lesions subsequently infiltrating to parenchyma. We describe a 52-year-old man with slowly progressive paresthesias and weakness in his legs. MRI showed diffuse leptomeningeal lesions throughout the brainstem and spinal cord without intraparenchymal lesions. A diagnosis of sarcoidosis was confirmed by cervical lymph node biopsy which detected noncaseating granuloma. Only leptomeningeal lesions throughout the brainstem and spinal cord could be observed in sarcoidosis.

Neuromyelitis optica (NMO) is presumably mediated by an autoantibody against aquaporin-4 densely expressed at the blood-brain barrier. In 18 patients with NMO, brain magnetic resonance imaging (MRI) findings were systematically reviewed. Brain MRI abnormalities were found for 89% of the patients, and the most prominent feature was "cloud-like enhancement," multiple patchy enhancing lesions with blurred margin, found in 90% of the patients with positive contrast enhancement. In NMO, brain MRI abnormalities are frequent, and cloud-like enhancement appears to be an MRI finding specific to NMO, possibly caused by primary involvement of the blood-brain barrier by the autoantibodies. © 2009 American Neurological Association.

A subgroup of limbic encephalitis is associated with antibodies against voltage-gated potassium channels (VGKC), and responds well to immuno-modulating therapies. Anti-VGKC antibodies are also found in Isaacs' syndrome and Morvan's syndrome, both of which are sometimes complicated by thymoma. We describe a 52-years-old man with limbic encephalitis, thymoma, and anti-VGKC antibodies, who presented with autonomic dysfunctions such as severe intestinal pseudo-obstruction, hyperhidrosis and hypertension. Thymectomy and corticosteroid therapy remarkably improved his symptoms. Brain magnetic resonance imaging showed hypothalamic lesions, in addition to the bilateral involvement of the medial temporal lobes. This patient had severe autonomic dysfunctions resembling those of Morvan's syndrome. This case may represent a subgroup of VGKC-antibody associated syndromes with a wide spectrum of symptoms, including Isaacs' syndrome, Morvan's syndrome, and limbic encephalitis. (C) 2008 Published by Elsevier B.V.

Intradural extramedullary tuberculoma of the spinal cord (IETSC) is a rare complication of tuberculosis, which can occur as a paradoxical response to antituberculous therapy. A 46-year-old woman with tuberculosis meningitis developed an acute sensory disturbance and paraplegia eight weeks after the antituberculous treatment was started. MRI revealed a cystic lesion at the Th 2 and 3 vertebrae levels, and continuous dural thickening. Laminectomy was performed; soft granulomas were unexpectedly observed inside the dura matter. After the operation, the patient experienced progressive improvement in motor strength. IETSC should be known as rare but possible complication of tuberculous meningitis.

The objective was to develop a simple method for evaluating putaminal atrophy in patients with the Parkinson variant of multiple system atrophy (MSA-P). We used magnetic resonance imaging to study 9 patients with MSA-P, 24 patients with cerebellar variants of multiple system atrophy (MSA-C), 38 patients with Parkinson's disease (PD), and 27 healthy control subjects. Posterolateral linearization of the putaminal margin was semiquantitatively scored and the putaminal area per intracranial area was calculated as the adjusted putaminal area. There was a negative correlation between the linearization scores and adjusted putaminal areas (r = -0.43, P < 0.001), such that the mean adjusted putaminal area in the group without putaminal linearization (0.0148 +/- 0.0022) was greater than that of the group with linearization (0.0124 +/- 0.0029, P < 0.005). Moreover, the occurrence of putaminal linearization was significantly higher in MSA-P patients (88.8%) than in MSA-C (8.3%), PD (7.9%) and healthy subjects (7.4%; P < 0.005). Putaminal linearization was a highly sensitive (0.89) and specific (0.91) measure for differentiating MSA-P. Our results suggest that evaluating posterolateral putaminal linearization is useful for assessing putaminal atrophy and for differentiating MSA-P from MSA-C, PD, and healthy subjects. (c) 2007 Movement Disorder Society.

Wolfram syndrome is a rare autosomal recessive disorder featuring diabetes insipidus, diabetes mellitus, optic atrophy, and deafness; DIDMOAD is a commonly accepted anonym for this disorder. We describe a 35-year-old man with Wolfram syndrome, who had marked atrophy of the brain stem, middle cerebellar peduncle, and cerebellum. Despite these MR imaging findings involving the pontocerebellar tract, the patient had no neurologic abnormalities suggesting dysfunction of the brain stem or cerebellum. Patients with Wolfram syndrome may have discrepancies between neurologic and radiologic findings.

健常者3例(全例男性)を対象として、外肛門括約筋の随意収縮時のfunctional MRI(FMRI)を施行した。評価断面はT11-12椎体レベルの軸位断とした。外肛門括約筋の随意収縮を30秒間行い(ON)、引き続き30秒間の安静(OFF)を行った。ON-OFFを計3回繰り返し、各断面での信号変化を測定し、T検定で有意差が出た部位をOFF時の画像上に表示した。結果として3例中2例で、外肛門括約筋の随意収縮時にT11-12椎体レベルの脊髄の賦活が認められた。また1例では大腿四頭筋や前脛骨筋の随意収縮時のFMRIを施行し、大腿四頭筋の収縮時には椎体内に広範な賦活部位が認められたが、脊髄の賦活範囲は狭かった。前脛骨筋の収縮時には明らかな賦活は認められなかった。FMRIによって腰仙髄支配筋の随意収縮に関連する脊髄領域の賦活を可視化できる可能性が示唆された。

31歳女。起床時に頭痛、吐気と伴に両下肢が全く動かず救急搬送された。完全対麻痺、第7胸髄レベル以下の全感覚低下、両下肢腱反射の低下を認め、脊髄MRIではT2強調画像で延髄から円錘部に至る全脊髄に高信号域と著明な脊髄腫脹を認め、造影MRIでも多発性の造影増強効果を認め急性縦断性脊髄炎と診断した。急性縦断性脊髄炎が初発のシェーグレン症候群と考えた。ステロイドパルス療法1クール施行後プレドニゾロン内服を開始し、徐々に漸減した。治療開始後1ヵ月より感覚障害が改善し始め、約2ヵ月後より対麻痺が急速に改善して約3ヵ月後には独歩が可能となった。入院時よりの完全尿閉は治療3ヵ月後より少量の自尿が出来たが、現在間歇式自己導尿を継続している。

Uro-neurological assessment was performed in four patients with small-fiber neuropathy due to amyloidosis (2 transthyretin-type/2 immunoglobulin light-chain-type). Voiding difficulties were due to detrusor weakness and impaired bladder sensation. In two patients cholinesterase inhibition treatment caused urge incontinence, indicating detrusor denervation supersensitivity. The underlying mechanisms of urinary dysfunction seem to involve postganglionic cholinergic and afferent somatic nerves.

Previously no functional study has been available for the mechanism of constipation in familial amyloid polyneuropathy (FAP). We performed a gut function test in a 78-year-old woman with transthyretin-type FAP who had severe constipation. The gut function test showed a prolonged colonic transit time, a low anal pressure at rest and upon squeezing, loss of the spontaneous phasic rectal contractions, and weak abdominal strain. All these abnormalities suggested a peripheral autonomic dysfunction, corresponding to the enteric neuropathy that was confirmed upon autopsy.

Atopic dermatitis (AD) is frequently associated with intestinal and cervical lesions. Staphylococcusaureus produces many kinds of toxins, the bacterial superantigens. The detection rate of toxins was 80.1% from 196 S. aureus strains. Neurological examinations revealed abnormalities in 59 out of 81 AD patients. Cervical magnetic resonance imaging (MRI) was performed in 46 patients randomly and showed abnormal findings in 38 of these patients. In 23 patients who underwent MRI and duodenal biopsy, 3 were found to be normal neurologically and 2 patients showed normal duodenal tissue. However, 18 patients had abnormal findings both on neurological examination and in duodenal tissue. Serial duodenal biopsy tests were performed in 10 AD patients. In 5 patients, the findings of chronic duodenitis disappeared after the therapy with povidone-iodine. These data indicate that the therapy was effective not only for the skin lesions, but improved gastrointestinal tract lesions and cervical myelopathy, by eradicating bacterial superantigens. Copyright (c) 2006 S. Karger AG, Basel.