Deep Brain Stimulation for the Treatment of Juvenile Parkinsonism: Case Report

Juvenile parkinsonism (JP) is characterized by the clinical manifestation of Parkinson syndrome before the age of 21 years old. This entity is often associated with genetic mutations. After all the possibilities of clinical treatment have been exhausted, surgical treatment is recommended, performed via deep brain stimulation (DBS) in the subthalamic nucleus (STN) or in the internal segment of the globus pallidus (GPi). The present study aimed to report the case of a patient with JP who underwent DBS in the STN with good clinical response. Neuromodulation via DBS is an option for the treatment of JP. However, since this entity is very rare, and even more peculiar when treated surgically, more studies are necessary to evaluate DBS used to control refractory manifestations and levodopa-induced dyskinesia, as well as surgical complications that may occur, aiming to gather more knowledge of the surgical management of JP. Despite the dysarthria after the DBS, the patient presented a satisfactory response regarding the symptoms, corroborated by the Parkinson's Disease Questionnaire (PDQ-39) score, which was 61.19% before the procedure, and decreased to 21.05% 14 months after the DBS.

Forma intermediária de síndrome de Foix-Chavany-Marie / síndrome de Worster-Drought associada a movimentos involuntários: aspectos neuropsicológicos e fonoaudiológicos / Intermediary form of Foix-Chavany-Marie / Worster-Drought syndromes associated to involuntary movements: neuropsychological and phonoaudiological features

A síndrome de Foix-Chavany-Marie (SFCM) caracteriza-se por apraxia da fala associada à paralisia bilateral da face, palato mole, língua e musculatura da faringe, mas com preservação das funções reflexas e automáticas. Na síndrome de Worster-Drought (SWD), há predomínio da disartria. Descrevemos o caso de uma jovem de 18 anos, que apresenta os achados clínicos e radiológicos compatíveis com a forma intermediária de SFCM/SWD, acompanhados de movimentos involuntários (coréia e distonia), fato de ocorrência rara na descrição destas síndromes.

Isolated dysarthria-facial paresis syndrome: a rare clinical entity which is usually overlooked.

BACKGROUND AND AIMS: The aim of the study is to search the lesion localization of the pure isolated facial paresis-dysarthria syndrome in patients who were admitted to our neurology clinic in a prospective study. METHODS: Over a period of six years, the patients who had no prominent sensorimotor dysfunction were examined by neurologists and underwent computerized tomography (CT) and/or magnetic resonance imaging (MRI). RESULTS: Eleven patients out of more than 2000 had the aforementioned clinical picture. Lacunar infarctions were identified at the corona radiata in nine patients, and at the internal capsule in two patients. As reported previously, facial paresis was usually mild and temporary. Six of our eleven patients were seen at the outpatient clinic one month later. Four of them had completely recovered and the other two had mild dsyarthria without any facial paresis. The other five could not be reached after leaving the hospital. CONCLUSIONS: Dysarthria-facial paresis is a rare clinical entity and possibly a variation of dysarthria-clumsy hand syndrome, and we suggest that pure facial paresis (FP) and pure dysarthria should be considered as very extreme examples of this syndrome.

Episodic ataxia: a case report and review of literature.

This report describes the clinical features of a 29 year female presenting with a 3 years history of episodes of cerebellar ataxia, dysarthria and nystagmus lasting 3-5 days, recurring almost every month. Sleep disturbance and buzzing in ears were noted 3-4 days before each episode. No other precipitant factor was present. Family history was negative. She was diagnosed as a case of episodic ataxia type-2 and was successfully treated with acetazolamide, a carbonic anhydrase inhibitor. She was asymptomatic at 2 year followup.

Esclerosis concéntrica de Baló: caso clínico / Balo concentric sclerosis: report of one case

We report a 50 years old woman admitted to the hospital due to progressive dysphagia and disarthria of there weeks duration. On admission a right hemiparesis was noted. CSF examination showed a protein of 9 mg/dl and no cells. A brain CAT scan showed rounded bilateral subcortical frontoparietal hypodense zones peripheral contrast material enhancement. Pseudobulbar palsies and hemiparesis worsened and the patient required nasoenteral feeding. She was discharged after four months, with severe dissabilities, with the diagnosis of Balo concentric, a progressive demyelinating disease