第137回日本神経学会東海北陸地方会東海北陸地方会 金沢 2013年11月2日

  1. Marburg Variant of Multiple Sclerosis(Acute MS)の一剖検例

29歳女性、発症3か月で死亡:この症例はすでにInternal Medicineに報告されていた。

Suzuki M, Kawasaki H, Masaki K, Suzuki SO, Terada T, Tsuchida T, Tokuyama T, Kono S, Komori T, Baba S, Kira J, Miyajima H. An autopsy case of the Marburg variant of multiple sclerosis (acute multiple sclerosis). Intern Med. 2013;52(16):1825-32.


We herein report an autopsy case of the Marburg variant of multiple sclerosis (MS). A 29-year-old woman developed acute and progressive neurological symptoms. A diagnosis of MS was suspected based on the patient’s clinical background and brain MRI findings and the lack of evidence of malignancy on a brain biopsy. Despite the administration of typical treatment for MS, a fatal outcome occurred three months after disease onset. The autopsy revealed multiple inflammatory demyelinating lesions in the central nervous system. In addition, two noteworthy histopathological features were observed compared with prototypical MS. We evaluate the pathogenic differences between the Marburg type and prototypical MS by discussing the neuropathology and cerebrospinal fluid (CSF) findings of our case.

  1. 当初てんかんと診断された成人発症SSPEの1例



Adult onset subacute sclerosing panencephalitis: clinical profile of 39 patients from a tertiary care centre


Clinical and laboratory characteristics of 39 patients with adult onset subacute sclerosing panencephalitis (SSPE) are described and compared to those of juvenile onset patients regarding preceding measles, age at onset, gender, interval between onset and diagnosis, clinical profile, and course during follow up. Diagnosis was based on clinical and electroencephalographic findings and raised anti-measles antibody titres in cerebrospinal fluid. Mean age at SSPE symptom onset was 20.9±4.9 years and mean interval from onset to diagnosis was 6.3±9.6 months. Referral diagnosis was accurate in only 12 patients. Presenting symptoms included myoclonus, behavioural changes, seizures, and cognitive, visual, and extrapyramidal disturbance. All patients received symptomatic therapy; 19 also received disease modifying agents. Five of seven pregnant women had successful deliveries. The follow-up period varied widely (maximum 60 months, median 9 months). The profile of adult onset SSPE did not differ from the rest of the cohort, except for a longer interval between measles infection and symptom onset (p<0.0001). SSPE in adults poses diagnostic challenges for clinicians. A high index of suspicion and appropriate investigations are necessary for early diagnosis and counselling.

Review Subacute sclerosing panencephalitis




Diffusion-Weighted MR Imaging Characteristics of an Acute Strokelike Form of Multiple Sclerosis


The characteristics of multiple sclerosis (MS) lesions on diffusion-weighted sequences and apparent diffusion coefficient (ADC) mapping at the very early phase of symptoms have not been clearly described. We report the case of a young woman who presented with a sudden pseudostroke form of MS resulting in hemiplegia and sudden aphasia. MR imaging showed a lesion of the left internal capsule with reduced ADC, which suggests an ischemic stroke. This case shows that very acute MS lesions may have reduced ADC on MR imaging, reflecting cytotoxic and not vasogenic edema.


再発性乳癌に合併した抗AQP4抗体陽性paraneoplastic neuromyelitis optica(NMO)の1例


Aquaporin-4 Autoantibodies in a Paraneoplastic Context






Aβ-related angiitis: Comparison with CAA without inflammation and primary CNS vasculitis.  October 29, 2013 81:1596-1603; published ahead of print September 27, 2013

Aβ-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy


Cerebral Amyloid Angiopathy-related Inflammation Presenting with Steroid-responsive

Higher Brain Dysfunction: Case Report and Review of the Literature


(橋詰良夫先生との共著が引用されている。Hashizume Y, et al: A 65-year-old man with headaches and left homonymous hemianopsia. Neuropathology 2004, 24(4):350-353.)


両側上肢近位筋の筋力低下を呈したflexion myelopathy症例

安城更生病院神経内科 佐野雨佳、杉浦真、安藤哲朗ほか


頸部前屈MRIT1強調画像では、C4/5に屈曲の頂点があり、C3-6に硬膜管後方の内椎骨静脈叢のうっ血を認めた。頭部前屈位CT myelographyでは、C4/5椎間レベルを中心とした硬膜前方移動が認められた。本症例の脊髄障害は若年性上肢遠位部髄節性筋萎縮症と同一病態の可能性があり、最大前屈部位が通常のC6椎体より吻側に偏するため、上位のC5,6









Hirayama K, Toyokura Y, Tsubaki T. Juvenile muscular atrophy of unilateral upper extremity; a new clinical entity. Psychiat Neurol Jap 1959; 61:2190-7.

Sobue I, Saito N, Iida M, Ando K. Juvenile type of distal and segmental muscular atrophy of upper extremities. Ann Neurol 1978; 3:429-432.

Seventy-one cases of distal and segmental muscular atrophy of the upper extremities with juvenile onset were studied. The clinical features consisted of: juvenile onset, male preponderance, unique distribution of the muscular atrophy in the hand and forearm, tendon reflexes hypoactive in most cases but hyperactive in some, no definite sensory disturbances, no involvement of the cranial nerves, and autonomic nerve disorders in the affected region. There was rapid progression during the 2 to 3 years after onset with a slowly progressive course thereafter. There were no abnormal laboratory findings except for electrophysiological and morphological findings of the affected muscles. The site of lesion was surmised to be from the C5 to T1 spinal segments with intramedullary involvement. The cause is unknown.

Mukai E, Sobue I, Muto T, Takahashi A, Goto S. Abnormal radiological findings on juvenile-type distal and segmental muscular atrophy of upper extremities. Rinsho Shinkeigaku 1985; 25:620-626.  若年性上肢遠位部髄節性筋萎縮症のレ線学的異常所見

Mukai E, Matsuo T, Muto T, Takahashi A, Sobue I. Magnetic resonance imaging of juvenile-type distal and segmental muscular atrophy of upper extremities. Rinsho Shinkeigaku 1987; 27:99-107.  若年性上肢遠位部髄節性筋萎縮症の MRI

Hirayama Disease: MR Diagnosis


Hirayama Flexion Myelopathy: Neutral-Position MR Imaging Findings—Importance of Loss of Attachment


Research Question 8








marugametorao について

神経内科専門医 neurologist
カテゴリー: 神経内科医, 神経学, 健康, 医学, 医学教育, 挑戦 パーマリンク



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