1.250 Cervical dystonia due to cerebellar stroke

Poster Presentations: Related Disorders 1.217 Late post-poliomyelitis syndrome with secondary parkinsonism A. Ilinca1° , M. Macovei 1 Romania

Objective: The authors studied 7 cases with poliomyelitis developed in childhood, with a sequellar peripheral motor neuron syndrome that complicated with extrapyramidal symptoms after approximately 30 years. The patients had an average age of 30, 3 men and 4 women, 3 cases with symmetrical Parkinson syndrome and 4 cases with unilateral Parkinson syndrome. Results: The authors consider the probability of a toxic, immunoallergic mechanism, interfering with molecular pathogenic pathways (alphasynuclein, parkin, ubiquitin carboxi-terminal hydrolase L1), with secondary oxidative stress and apoptosis that explain the demyelination seen on MRI. Late SLA symdromes are already known as part of the late postpoliomyelitis syndrome, but we didn’t find in the consulted literature Parkinson-like syndromes with a possible immunological mechanism and inflammatory phenomena. Methods: All the patients studied had increased values of albumin in the CSF, of about 1 g/dl, and significantly elevated levels of IgG. Cerebral MRI showed areas of demyelination in the basal ganglia, prevailing in the globus pallidus. Conclusion: We propose to monitor the IgG levels and the influence of IVIG therapy on clinical aspect improvement in patients with late postpoliomyelitis syndrome and extrapyramidal manifestations.

Related Disorders 1.250 Cervical dystonia due to cerebellar stroke M. Habek1° , V. Brinar, I. Zadro, V. Djakovic Croatia

1 Zagreb,

Objective: It is thought that site of pathologic involvement in patients with dystonia is in the basal ganglia. However, recently there have been suggestions that cerebellum plays a key role in the development of dystonia. Method: We describe a patient who developed cervical dystonia after ipsilateral cerebellar infarction in the territory of superior cerebellar artery (Figure 1). Two days after symptoms onset she developed abnormal posturing of her head, with sustained involuntary contractions of the cervical muscles so the head was rotated to the left and down. Treatment with clonazepam and baclofen was introduced with significant improvement of symptoms.

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Results: There are several characteristsics of this case which made us to conclude that there is casual relationship between these two entities. Firstly, the onset of dystonia occurred almost simultaneously with the onset of left cerebellar syndrome. The facts that there was actively contraction of the right sternocleidomastoid muscle with hypertrophy, no signs of dystonic movements on other parts of the body after follow-up, no signs of other structural lesions in the brain except infarction in the cerebellum, no family history of dystonia and patient did not take any neuroleptic drugs strongly support that this is a case of secondary dystonia due to a structural lesion of the left cerebellar hemisphere. It is possible that destruction of olivocerebellar pathway caused abnormal output of Purkinje cells and thus caused dystonia. Conclusion: This report together with other cases strongly supports the hypothesis of cerebellar involvement in pathogenesis of dystonias.

1.251 The optimal settings of pallidal deep brain stimulation for DYT1-positive primary generalized dystonia R. Okiyama1° , F. Yokochi, N. Izawa, M. Taniguchi, T. Terao, T. Kawasaki, H. Takahashi 1 Tokyo, Japan Objective: Deep brain stimulation (DBS) of the globus pallidus internus (GPi) shows promising efficacy in the treatment of severe primary generalized dystonia. However, the optimal stimulation settings are uncertain. To decide the optimal stimulation settings in patients with DYT1-positive idiopathic primary generalized dystonia. Method: Patients; 7 with DYT1-positive primary generalized dystonia, treated with GPi DBS in our institution (2001–2005). Clinical Evaluation: the Burk–Fahn–Marsden scale (BFM scale) before and after GPi DBS. Anesthesia: generalized anesthesia, using propofol. Operative procedure: implant the electrode (model 3387 or 3389) for stimulation bilaterally, using both MRI and electrophysiological guidance. Results: All of 7 patients dramatically improved by our stimulation settings of which were the same as those in patients with Parkinson’s disease (60/90 ms. 130/160 Hz, 1.5−3.6 V). % improvement of Burk–Fahn–Marsden scale in our institution was almost the same as those in literatures using stimulation with higher frequencies (130–250 Hz), wider width (around 210 Hz) and higher voltage (higher than 2.5 V). Conclusion: Stimulation settings same as in patients with Parkinson’s disease (60/90 ms. 130/160 Hz, 1.5−3.6 V) seem to be sufficiently effective for idiopathic primary generalized dystonia. The optimal settings of pallidal deep brain stimulation for idiopathic primary generalized dystonia are open to argument.

1.252 Clinical characterization of a large Dutch family with primary focal dystonia M.F. Contarino1° , E. Berger-Plantinga, E. Foncke, F. Baas, J.D. Speelman, M.A.J. Tijssen 1 Amsterdam, Netherlands Objective: We describe a large Dutch family, affected by primary focal dystonia. The family originates from a small village, on a former island. Method: Two movement disorders neurologists examined all the family members who agreed to participate. A complete neurological evaluation was performed on each subject, standardized videos were made, and blood samples were collected for DNA analysis. Two other movement disorders neurologists evaluated the videos independently. Subjects were classified as “affected”, “possibly affected” or “not affected”. A diagnosis was defined if all 4 neurologists agreed on the definition. When there was no agreement on the diagnosis, the lowest mentioned grade was chosen. Each neurologist performed dystonia rating scales for all the subjects defined as “affected” or “possibly affected”. Analyses of DYT1 and e-sarcoglican genes were carried out.