Biomedical subjects
Publications and source records attributed to Kiyoshi Negoro.
A 17-year-old boy without a significant past medical history presented with recurrent cluster-like headaches induced by meals for 3 years. Magnetic resonance images showed a pituitary tumor. Just after starting treatment with cabergoline, the headaches resolved completely and the patient has been absolutely free from such headache attacks for 2 years.
Pseudomigraine with pleocytosis (PMP) is an uncommon disease in Japan. The diagnostic criteria include at least one episode of transient neurological deficit accompanied or followed by migraine-like severe headache, cerebrospinal fluid (CSF) lymphocytosis, and normal neuroimaging. Both the etiology and the pathophysiology of PMP is not yet well defined. We report a 40-year-old man with a PMP-like syndrome. He came to our clinic because of severe throbbing headache and amnesia, and the examination showed CSF lymphocytosis of 23/mm3, a transient decrease of cerebral blood flow in the left thalamus. All the symptoms were completely resolved within 2 months.
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Corticobasal degeneration (CBD) and atypical progressive supranuclear palsy (PSP) were reviewed with special reference to their symptomatology, laboratory examination and differential diagnosis. In our survey of the autopsy cases of CBD in Japan, only about 60% of the pathologically confirmed CBD cases were correctly diagnosed clinically, meaning that atypical (non-classical) clinical forms are common in CBD. Concerning the autopsy cases of PSP in Japan, 75% of the PSP cases had correct clinical diagnosis. In literatures, the clinically atypical CBD includes (1) frontotemporal dementia, also with primary progressive aphasia and frontal lobe dementia as subforms, (2) PSP-like form, and (3) others. The clinically atypical PSP comprises (1) pure akinesia, (2) pure easy falling syndrome (Yuasa), (3) no postural instability, (4) no gaze palsy, (5) asymmetric parkinsonism, (6) no or severe dementia, etc.. PSP with cortical manifestations such as primary progressive aphasia and CBD-like features were also reported. The atypical CBD and PSP probably reflect the distribution of tau pathology different from that in typical forms. Except for the report that phosphorylated tau is increased in CSF in CBD, but not in PSP (Urakami et al), no reliable laboratory data have been available on clinical differentiation between atypical CBD and PSP.
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We conducted the survey of patients with CBD in 2001 for 29 neurological institutions in Japan which joined Research Group on Neurodenerative Diseases supported by Ministry of Health and Welfare. Provisional diagnostic criteria of CBD were as follows: "probable CBD" means a clinical category including (1) classical form, consisting of progressive limb-kinetic apraxia and akinetic rigidity, predominant on one side, associated with late dementia, (2) quasi-classical form, revealing other corticobasal signs, predominant on one side, associated with late dementia, and (3) non-classical from, showing early aphasia, dementia, behavior disorder, etc., followed by lateralized limb-kinetic apraxia and akinetic rigidity. "Possible CBD" was not made. "Definite CBD" is pathologically confirmed CBD. There were 151 patients with "probable CBD", comprising 121 patients with classical form, 17 with quasi-classical form and 13 with non-classical from, while patients with "definite CBD" were 13. The number of patients with progressive supranuclear palsy (PSP) was also examined. The ratio of the number of patients CBD/PSP was 1/2.6 in clinical cases and 1/2.5 in autopsy cases. Nakashima et al. performed population survey of PSP patients in Yonago city in 1999, demonstrating prevalence of PSP 4.36/100,000. Considering this rate, the prevalence of CBD is assumed as 1.7 and the presumed number of PSP and CBD patients in 1999 in Japan could be 5,500 and 2,100, respectively.