Rinshō shinkeigaku = Clinical neurology
-
Review Case Reports
[Clinical features and mechanisms of chronic migraine and medication-overuse headache].
Chronification of migraine headaches is one of the most urgent issues. Chronic migraine (CM) and medication overuse headache (MOH) are defined in international classification of headache disorders II (ICHD-II). Appendix criteria of CM and MOH were submitted and will take over the original criteria. ⋯ Apart from medication overuse, there have been reported some new risk factors for migraine chronification, including frequent headache, female sex, obesity, low income, low education, stress by life events, depression, snoring, sleep disorders, and past history of neck or head injury. Chronification of migraine severely disturbs the quality of patient's life. More attention should be paid and the further and extensive studies are urgently necessary.
-
Human prion diseases are classified into 3 categories according to etiologies: idiopathic of unknown cause, acquired of infectious origin, and genetic by PRNP mutation. The surveillance committee have analyzed 2,494 cases and identified 1,402 as prion diseases. Most of them are idiopathic, namely sporadic CJD (77%) with less genetic and acquired prion diseases (17% and 5%, respectively). ⋯ We also have some incidents in which brain surgery was done before the diagnosis of prion disease and many other patients were operated using the same operating instruments before their sterilization against prion disease had been done. The explanation of possibility of prion disease infection to the patients and their follow-up was started by the incident committee. It is very important for all the nations to cooperate with each other in order to overcome this intractable disease.
-
Multiple sclerosis (MS) is a demyelinating disease of the central nervous system (CNS) while neuromyelitis optica (NMO) is an inflammatory disease of the CNS that selectively affects the optic nerves and spinal cord. Recently, a specific IgG against NMO, designated NMO-IgG, was discovered, and the relevant antigen was found to be aquaporin 4 (AQP4), one of the major water channel proteins in the CNS. The sensitivity of NMO-IgG/anti-AQP4 antibodies for NMO varies from 30% to 80%, while specificity is 90-100%. ⋯ None of six other patients with magnetic resonance imaging-confirmed Baló's disease was seropositive for anti-AQP4 antibodies. I therefore propose that AQP4 astrocytopathy, in the absence of anti-AQP4 antibodies, is characteristic of Baló's disease. Since a similar loss of AQP4 without perivascular deposition of immunoglobulin and complement is also observed in autopsied CNS tissues from NMO and MS cases, I consider that autoantibody-independent astrocytopathy may widely occur in human CNS demyelinating diseases, including Baló's disease, MS and NMO.
-
Wernicke's encephalopathy is a syndrome characterized by ataxia, ophthalmoplegia, and confusion with thiamine deficiency. We reported on two Japanese brothers with a newly discovered recessively inherited syndrome similar to Wernicke's encephalopathy that developed in the second decade of life; this syndrome was manifested clinically as thiamine-responsive diplopia, ataxia and confusion without serum thiamine deficiency. The patients had complex partial seizure. ⋯ Gene-expression analyses of mammalian culture cells showed that intracellular thiamine uptake activities were decreased significantly. High expression of SLC19A3 RNA in the thalamus may explain the selective thalamic lesions on MRI. The identification of this syndrome proves insight into the thiamine metabolism associated with Wernicke's encephalopathy in humans.