Interruption of deep brain stimulation of the globus pallidus in primary generalized dystonia

Stimulation (DBS) of the globus pallidus (GP) is effective to treat generalized dystonia. Little is known about the evolution of dystonia in case of arrest after a long period of stimulation. This study describes the course of dystonia during a 48 hours period without stimulation followed by a 24 hours period after turning ON the stimulator. 14 patients with generalized dystonia treated with bilateral GP DBS for 3 years or more were recruited. Blinded video‐based analysis was performed using Burke‐Fahn‐Marsden scale at (1) baseline (ON stimulation), (2) up to 48 hours after the stimulator was turned OFF, and (3) 24 hours after the stimulator was turned ON. 13 patients completed the 48 hours OFF‐stimulation period. The dystonia movement score progressively worsened from 24.3 ± 13.9 at baseline to 48.9 ± 19.8 after 48 hours (P < 0.00001). The disability score also worsened from 4.4 ± 1.2 at baseline to 5.7 ± 1.5 after 48 hours without stimulation (P < 0.001). When the neurostimulator was turned ON, the dystonia scores returned to baseline level after 10 hours. The interruption of GP DBS in dystonia results in a progressive worsening which is rapidly reversible once the neurostimulator is turned ON. © 2009 Movement Disorder Society     

Radiotherapy-induced pemphigus vulgaris with autoantibodies targeting a 110 kDa epidermal antigen

Background Pemphigus is an autoimmune intraepidermal blistering disease mediated by autoantibodies targeting desmosomes. It can be induced by many triggers, such as ionizing radiation. Methods We report a case of radiotherapy‐induced pemphigus (RIP) with a review of the published cases in the English and French literature. Results A 61‐year old man was diagnosed to have epidermoid carcinoma of the piriform sinus and then received a 70 Gy radiation therapy. One month after the treatment completion, multiple blisters and erosions occurred initially on the site of irradiation, then in other skin areas. Histological examination showed an intraepidermal blister with acantholysis and necrosis of individual keratinocytes. Direct immunofluorescence and indirect immunofluorescence were typical of pemphigus. Immunoblot revealed antibodies reacting with a 110 kDa antigen. This feature was consistent with the diagnosis of RIP. Less than 20 cases of RIP have been reported previously. Mean age at diagnosis was 64.2 years, and there is a slight female preponderance. RIP occurred, in most cases, initially within the area of irradiation. Conclusion Our patient showed some distinctive findings never reported previously in RIP: a histological focal keratinocyte necrosis, and the presence of autoantibodies reacting with a 110 kDa keratinocytic protein in immunoblot analysis. Because of a different prognosis, it is important to differentiate RIP and paraneoplastic pemphigus (PNP), although cases of ionizing radiation‐induced PNP had also been described. As in our patient, RIP seems to respond well to systemic corticosteroids and immunosuppressive therapy, which induce remission within a few months.     

Penile shaft involvement in pityriasis versicolor

The Malassezia yeasts are among the normal human cutaneous flora in adults. They are also reported as part of the microflora of male genital region, mostly in uncircumcised males. The prevalence of Malassezia yeast colonization on the glans penis of circumcised males is discussed in multiple studies. We report the case of a male patient with extensive pityriasis versicolor that does not involve the preputial space.     

Angiomatoid tumor of the thyroid gland: primitive angiosarcoma or variant of anaplastic carcinoma?

Thyroid gland angiomatoid tumors are an extremely aggressive neoplasms with varied histological patterns and features of endothelial differentiation. The histogenesis of thyroid angiomatoid tumors has been controversial for many years: these tumors may be either a variant of anaplastic carcinoma, or an angiosarcoma. We report a case of thyroid angiomatoid tumor in a 68-year-old woman. We also discuss, through a review of the literature, the pathologic criteria that could be used to distinguish between angiosarcoma and anaplastic carcinoma of the thyroid.