Obsessive-compulsive symptoms among patients with blepharospasm and hemifacial spasm.

OBJECTIVE: The aim of this study was to compare the prevalence and the severity of different obsessive-compulsive disorder (OCD) symptoms reported by patients with blepharospasm (BSP) with those reported by patients with hemifacial spasm (HFS). We hypothesized that, since patients with BSP present a dysfunctional striato-thalamo-cortical circuitry, they would exhibit higher prevalence and/or greater severity of OCD symptoms than patients with HFS, a condition that results from peripheral irritation of the facial nerve. METHODS: Twenty-two patients with BSP and 31 patients with HFS were systematically evaluated by means of a sociodemographic and clinical questionnaire, the Mini International Neuropsychiatric Interview, the Obsessive-Compulsive Inventory-Revised, the Beck Depression Inventory (BDI), the Beck Anxiety Inventory (BAI) and the Mini Mental State Examination (MMSE). Diagnostic groups were compared using the Mann-Whitney U test for continuous variables and the Pearson's goodness-of-fit χ(2) test for categorical ones; Fisher's Exact Test was employed when indicated. Correlations between continuous variables were evaluated by means of Spearman coefficients. RESULTS: Patients with BSP and HFS were not significantly different in terms of sociodemographic characteristics and most neuropsychiatric features. Nevertheless, while checking was associated with shorter duration of BSP (Spearman's rho=-0.54; P=.01), hoarding correlated with a longer duration of HFS (Spearman's rho=0.40; P=.04). Length of abnormal movements did not correlate with the BDI, BAI and MMSE scores. CONCLUSIONS: The finding that the severity of different OCD symptoms did not differ between the BSP and HFS groups suggests that BSP may not interfere significantly with behavioral components of the striato-thalamo-cortical circuitry. However, the fact that OCD symptoms were found to follow different courses in distinct diagnostic groups deserves further study.

Short-lived response of cervical dystonia to electroconvulsive therapy.

Cervical dystonia (CD) or spasmodic torticollis is the most frequent form of focal dystonia. Cervical dystonia is characterized by sustained neck spasms, abnormal head posture, head tremor, and pain. A 53-year-old male patient with the diagnosis of CD developed an episode of delusional depression and was treated with electroconvulsive therapy (ECT). An unexpected and dramatic improvement of CD was seen during the first 2 days after each ECT session. That therapeutic effect was not sustained and vanished soon afterward. The effectiveness of ECT for CD, although too brief to be recommended as a useful treatment, may shed light on the pathophysiology of this problematic movement disorder.

Myopathy of distal lower limbs: the clinical variant of Miyoshi.

Miyoshi distal dystrophy is a rare myopathy characterized by an autosomal recessive pattern of inheritance and it is prevalent in Japan. Onset of disease is in early adult life with weakness and atrophy of the leg muscles. Recently gene linkage to chromosome 2p12-14 has been established. We report three sisters, born of consanguineous parents. All of them noticed weakness and atrophy of leg muscles, and could not walk on their heels. In all of them the creatine kinase concentrations were very high. The electromyography showed myopathic patterns and the muscle biopsy disclosed dystrophic changes and an absence of dysferlin. There are few cases reported of Miyoshi distal dystrophy in Latin America. The Miyoshi myopathy may be distinct among the hereditary distal myopathies.