We report a 14-year-old girl who developed chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) during the course of myasthenia gravis. Myasthenia gravis, which was clinically of ocular type, but with waning phenomenon of the extremities, occurred at 2 years and 4 months of age. Muscle weakness of the lower extremities gradually developed over the next 6 years. The electrophysiological and pathological findings fulfilled the criteria of "possible CIDP" with severe axonal involvement. The signs of myasthenia gravis and CIDP fluctuated synchronously. A common immunological abnormality was suggested to underlie this rare association of myasthenia gravis and CIDP in childhood.
BACKGROUND:Adult patients with moyamoya disease (MMD) may present with mild cognitive dysfunction, even those without evidence of conspicuous brain parenchymal damage. This cognitive dysfunction might be caused by local frontal lobe ischemia.OBJECTIVE:To explore the relationship between frontal lobe hemodynamic insufficiency and cognitive dysfunction in patients with MMD.METHODS:Thirty adult patients with MMD without conspicuous brain parenchymal damage were retrospectively examined. Patients with MMD with frontal lobe intracerebral steal phenomenon on single photon emission computed tomography were defined as group S (n = 13) and those without it were defined as group P (n = 17). A comparative group comprising patients with unruptured intracranial aneurysm was defined as group C (n = 30). The results of various cognitive and intelligence tests and a composite cognitive score were compared between groups.RESULTS:The digit span test forward version (P = .041), frontal assessment battery (P = .022), and composite cognitive score (P = .015) z-scores were significantly lower in group S than group C. Adjusting for sex and age, patients in group S had a significantly lower composite cognitive score compared with those in group C in multiple regression analysis (P = .037). Executive dysfunction and working memory dysfunction may be involved in the cognitive decline observed in group S.CONCLUSION:Mild cognitive dysfunction in MMD was associated with frontal lobe hemodynamic insufficiency. Future studies should examine whether revascularization can improve cerebral hypoperfusion and neurocognitive function in these patients.
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