Effects of white matter lesions on brain perfusion in patients with mild cognitive impairment.

OBJECTIVE: To evaluate the effects of white matter lesions on regional cerebral blood flow in subjects with amnestic mild cognitive impairment. PATIENTS AND METHODS: Seventy-five subjects with mild cognitive impairment (36 men and 39 women; mean age, 78.1 years) were included in the study. We used the Mini-Mental State Examination to assess cognitive function. All subjects underwent brain magnetic resonance imaging and 99mTc ethylcysteinate dimer single photon emission computed tomography. Subjects were stratified based on the presence or absence of white matter lesions on magnetic resonance imaging. Statistical parametric mapping of differences in regional cerebral blood flow between the two groups were assessed by voxel-by-voxel group analysis using SPM8. RESULTS: Of all 75 subjects with mild cognitive impairment, 46 (61.3%) had mild to moderate white matter lesions. The prevalence of hypertension tended to be higher in subjects with white matter lesions than in those without white matter lesions. Mini-Mental State Examination scores were significantly lower in subjects with white matter lesions than in those without white matter lesions. Subjects with white matter lesions had decreased regional cerebral blood flow mainly in the frontal, parietal, and medial temporal lobes, as well as the putamen, compared to those without white matter lesions. CONCLUSION: In subjects with mild cognitive impairment, white matter lesions were associated with cognitive impairment and mainly frontal lobe brain function.

[A amyotrophic lateral sclerosis (ALS) 4 family misdiagnosed as hereditary spastic paraplegia-a case report].

We report a 44 years old man with slowly progressive muscular atrophy of the extremities for over 30 years. He experienced difficulty in walking in his 10's and was diagnosed as hereditary spastic paraplegia (HSP) in his 20's. And then, muscle atrophy of the extremities slowly progressed especially in his distal muscles. Sensory axonal neuropathy was detected with sural nerve biopsy. His father and uncle have been diagnosed as HSP in their early days. His father noticed weakness of his leg in his 20's. He lost motor function of the leg in his 60's. In addition, marked disturbance of thermal sensation, vibration, and sense of position were found by physical examination. Our genetic study detected senataxin (SETX) gene mutation (c.8C>T,p.T3I) in the blood of those two patients, and they had been identified as family cases of amyotrophic lateral sclerosis (ALS) 4. As clinical symptoms of ALS4 would be similar to those of HSP at the onset, we suggest considering ALS4 in seeing patients with HSP without gene diagnosis.

[A case of neurosarcoidosis with swelling and gadolinium enhancement of spinal nerve roots on magnetic resonance imaging].

An 80-year-old woman was admitted to our hospital because of developed sense of constriction in the trunk and gradually progressive numbness and muscle weakness in the upper and lower extremities. Cerebrospinal fluid analysis showed increased cell count and protein level. Gadolinium enhanced magnetic resonance imaging (MRI) of spine showed the enhancement and swelling of bilateral nerve root in the cervical and lumbar segments. Although chest computed tomography showed neither bilateral hilar lymphoadenopathy nor lung lesions and serum angiotensin converting enzyme and lysozyme (ACE) were normal, tuberculin skin test was negative and cell count and CD4/CD8 elevated in bronchoalveolar lavage fluid. Biopsy specimen of scalene lymph node showed noncaseating granuloma. The patient was treated with oral predonisolone, which improved her symptoms and abnormalities on MRI. It is important to consider neurosarcoidosis in the differential diagnosis of polyradiculopathy with swelling and gadolinium enhancement of spinal nerve roots.

[Case of Vogt-Koyanagi-Harada disease associated with non-herpetic acute limbic encephalitis with autoantibodies against glutamate receptor epsilon2 in the cerebrospinal fluid].

We report a case of Vogt-Koyanagi-Harada (VKH) disease associated with non-herpetic acute limbic encephalitis with autoantibodies against glutamate receptor epsilon2 in the cerebrospinal fluid. A 42-year-old woman developed a complaint of visual distortion, visual disturbance, headache and mild psychiatric symptoms, such as anxiety and depression. She was diagnosed as VKH through the fidings of fluorescein fundus angiography, which revealed patchy hypofluorescence associated with delayed choroidal filling at early fluorescein angiographic phase, and spotted choroidal hyperfluorescence and pooling of dye at late phase. Analysis of the cerebrospinal fluid (CSF) showed slight increase of leukocyte count (49/microl, mononuclear cells) and immunoglobulin (Ig) G index. An anti-GluRepsilon2 IgM antibody was positive in CSF. Brain magnetic resonance imaging (MRI) showed a monofocal hyperintensity lesion in the left parahippocampal gyrus on T2-weighted and fluid-attenuated inversion recovery (FLAIR) images. We diagnosed her VKH disease associated with non-herpetic acute limbic encephalitis. She was treated with oral prednisone, 70 mg day and her symptoms have gradually improved. To our knowledge, meningoencephalitis in VKH disease is extremely rare and the analysis of anti-GluRepsilon2 IgM antibody in CSF has not been reported. We speculate that a certain immunologic mechanism, including the anti-GluRepsilon2 IgM antibody, contributes to the pathogenesis of the VKH disease with non-herpetic acute limbic encephalitis.

Primary central nervous system lymphoma with cortical laminar hemorrhage.

Here we report a case of primary central nervous system (CNS) lymphoma with cortical laminar hemorrhage. The present case showed an acute onset of focal neurologic signs and bilateral cortical lesions surrounded by peripheral white matter edema on magnetic resonance imaging. A part of the left frontal cortical lesion was hyperintense on T1-weighted images and hypointense on T2-weighted and T2-weighted gradient-echo images, suggesting subacute laminar hemorrhage. The patient was initially diagnosed with multiple hemorrhagic infarctions, but a biopsy specimen revealed diffuse large B-cell lymphoma with hemosiderin deposits. Immunohistochemical studies revealed that the tumor cell cytoplasm and membrane stained positively for anti-vascular endothelial growth factor antibody. The present case reconfirms the danger of making a specific lymphoma diagnosis based on magnetic resonance imaging findings alone and that histopathologic examination following brain biopsy is necessary for a correct diagnosis. Vascular endothelial growth factor expression might be associated with the intratumoral hemorrhage.