杉山 淳比古

A 61-year-old woman presented with a 10-month history of gait disturbance and a 7-month history of urinary incontinence. The Hasegawa dementia scale-revised score indicated cognitive impairment. Brain magnetic resonance imaging (MRI) indicated hydrocephalus with disproportionately enlarged subarachnoid space. This is usually considered a characteristic finding in idiopathic normal pressure hydrocephalus (iNPH). Ventriculo-peritoneal shunting improved the patient's symptoms. Neurosarcoidosis was suspected as a cause of the hydrocephalus because of the abnormalities in the cerebrospinal fluid and the abnormal enhancement of the cauda equina, the leptomeninges of the brainstem, and the spinal cord, as seen on MRI with gadolinium enhancement. A biopsy from the mediastinum lymph nodes confirmed the histological diagnosis of sarcoidosis. Physicians should consider the possibility of neurosarcoidosis in patients presenting with hydrocephalus, even in cases where clinical and radiological data are characteristic of iNPH.

Background: MSA is clinically classified into two phenotypes: parkinsonism predominant (MSA-P) and cerebellar ataxia predominant (MSA-C). However, little is known about the differences in urinary dysfunctions between MSA-C and MSA-P. We investigated the differences in urinary and cardiovascular dysfunctions between MSA-C and MSA-P.Methods: We retrospectively reviewed the medical records of patients with MSA diagnosed as having probable or possible MSA according to Gilman's second consensus criteria from January 2007 to September 2013 in our hospital. Data regarding the initial symptoms, onset of urinary symptoms, and results of urodynamic (including anal sphincter electromyography) and head-up tilt tests were collected.Results: A total of 100 patients with MSA, including 59 patients with MSA-C and 41 with MSA-P, were reviewed. Initial symptoms were autonomic (n = 12) and cerebellar (n = 47) in the MSA-C phenotype and were autonomic (n = 14) and parkinsonian (n = 27) in the MSA-P phenotypes. Urodynamic study indicated that bladder contraction was more severely impaired in patients with MSA-P than in those with MSA-C. In the head-up tilt test, the decrease in diastolic blood pressure was significantly larger in the MSA-P phenotype than that in the MSA-C phenotype. Acontractile bladder during the pressure flow study increased likelihood that the phenotype is MSA-P (odds ratio: 6.67; 95% confidence interval: 1.004-44.284; P = 0.050).Conclusions: Urinary dysfunctions were more severe in MSA-P compared with MSA-C. Detailed urodynamic study was helpful for detecting subtle differences between MSA-C and MSA-P.

Trigeminal root entry zone abnormality on brain magnetic resonance imaging has been frequently reported in multiple sclerosis patients, but it has not been investigated in neuromyelitis optica patients. Brain magnetic resonance imaging of 128 consecutive multiple sclerosis patients and 46 neuromyelitis optica patients was evaluated. Trigeminal root entry zone abnormality was present in 11(8.6%) of the multiple sclerosis patients and two (43%) of the neuromyelitis optica patients. The pontine trigeminal root entry zone may be involved in both multiple sclerosis and neuromyelitis optica. (C) 2015 Elsevier B.V. All rights reserved.

To study distribution and patterns of nerve hypertrophy in chronic inflammatory demyelinating polyneuropathy (CIDP), magnetic resonance neurography with 3-dimensional reconstruction of short tau inversion recovery images was performed in 33 patients. This technique clearly showed longitudinal morphological changes from the cervical roots to the nerve trunks in the proximal arm. Nerve enlargement was detected in 88% of the patients. According to the clinical subtype of CIDP, typical CIDP patients showed symmetric and root-dominant hypertrophy, whereas Lewis-Sumner syndrome patients had multifocal fusiform hypertrophy in the nerve trunks. The patterns of nerve hypertrophy presumably reflect the different pathophysiology of each CIDP subtype. Ann Neurol 2014.

Objective: To identify useful MRI abnormalities in the putamen for diagnosing multiple system atrophy. Methods: Patients with multiple system atrophy (n = 15), Parkinson's disease (n = 16), or progressive supranuclear palsy (n = 9) and healthy controls (n = 10) were enrolled. Using a visual analog scale, 4 examiners independently rated high-intensity signals along the lateral putamen on T2-weighted and T2*-weighted images, low-intensity signals within the putamen on T2-weighted and T2*-weighted images, and putaminal atrophy. Receiver operating characteristic analyses were performed, and the area under the receiver operating characteristic curve was calculated. Results: For differentiating multiple system atrophy from progressive supranuclear palsy, Parkinson's disease, and healthy controls, the mean area under the curve values was the highest for low-intensity signals within the putamen on T2*-weighted images (0.797, 0.867, 0.896, respectively). Variations in the area under the curve values among the 4 examiners were the smallest in low-intensity signals within the putamen on T2*-weighted images. Good inter-rater reliability was achieved for low-intensity signals within the putamen on T2*-weighted images and high-intensity signals along the lateral putamen on T2*-weighted images. Conclusion: Low-intensity signals within the putamen on T2*-weighted images is the most useful MRI abnormality for diagnosing multiple system atrophy. (C) 2015 Elsevier B.V. All rights reserved.

Myeloid sarcoma is a rare hematological disorder that presents as an extramedullary mass of immature myeloid precursors. We herein present the case of a 57-year-old man with a seven-month history of progressive weakness in the right upper extremity. Reconstruction magnetic resonance neurography showed a marked enlargement of the right brachial plexus. Fluorodeoxyglucose positron emission tomography revealed a radioactive lesion in the sacrum, in addition to the right brachial plexus, and a biopsy of the sacrum revealed myeloid sarcoma. The brachial plexus lesion was also regarded as myeloid sarcoma because of the treatment response. Isolated myeloid sarcoma involving the brachial plexus is very rare and its diagnosis is difficult as there was neither a history of leukemia nor bone marrow involvement in this patient. In this case, reconstructed magnetic resonance neurography was useful for detecting the brachial plexus mass lesion which led to an early diagnosis and good recovery.

Helicobacter cinaedi, a gram-negative spiral bacillus that inhabits the intestinal tracts of rodents and primates, is associated with gastroenteritis in humans. H. cinaedi infection has been commonly reported in immunocompromised individuals such as human immunodeficiency virus-infected patients, but rarely in immunocompetent individuals. Prior contact with animals has attracted attention as a possible Source of H. cinaedi infection. We report a case of meningitis in an immunocompetent 34-year-old woman who had daily contact with a kitten for a month. She developed acute headaches, fevers, and chills. Neurological examination revealed neck stiffness and her cerebrospinal fluid (CSF) exhibited polymorphonudear pleocytosis and a decreased concentration of glucose. Blood and CSF cultures were negative; however, the pathogen responsible for her condition was identified as H. cinaedi by polymerase chain reaction in CSF. This is the first adult case of meningitis caused by H. cinaedi. Thus, this bacillus should be considered a possible causative agent of bacterial meningitis in healthy adults. (C) 2013 Published by Elsevier B.V.

A 72-year-old man presented with dizziness and left hand muscle atrophy. Magnetic resonance imaging revealed a spinal cord cavity and descent of the cerebellar tonsils. His diagnosis was Chiari I malformation with syringomyelia. No cerebellar signs were observed on physical examination. The cause of dizziness was investigated using a video-based eye movement tracker, which revealed a downward smooth pursuit velocity gain significantly below normal when expressed relative to the horizontal pursuit velocity gain. Vestibulocerebellar damage can cause mild downward pursuit deficit. The downward to horizontal smooth pursuit velocity gain ratio may be a more sensitive means of detecting vestibulocerebellar damage early.