Nilson Becker

Increased Frequency and Range of Sexual Behavior in a Patient with Parkinson's Disease After Use of Pramipexole: A Case Report

INTRODUCTION Several recent reports have linked the use of dopamine agonists (DAs) to a variety of compulsive behaviors in patients with Parkinsons disease (PD). These inappropriate behaviors may include pathological gambling, compulsive shopping, and hypersexuality. AIM To report the case of a patient with increased range of sexual behavior after use of pramipexole, a DA. METHODS A 67-year-old man with a 7-year diagnosis of PD treated with levodopa and pramipexole presented with a dramatic change in sexual behavior after an increase in DA dose. RESULTS The patient, who historically was a very shy and conservative person, started to present increased frequency of sexual intercourse with his wife, during which he began speaking obscenities with an extreme preference for anal intercourse, preferences never requested before. After pramipexole was withdrawn, complete remission was observed with return to his usual sexual behavior. CONCLUSIONS Hypersexuality and paraphilias are complications not uncommonly found in patients with PD under dopaminergic treatment. Further studies are needed for the understanding of this complex complication, and particularly the most prevalent relationship between pathological hypersexuality and use of DAs.

Cervical dystonia: clinical and therapeutic features in 85 patients

We studied patients with cervical dystonia (CD) to determine clinical features and response to botulinum toxin A (BoNT/A). Patients were submitted to clinical, laboratory and neuroimaging evaluation. BoNT/A was injected locally in 81 patients using electromyographic guidance. Four patients who had had previous treatment were considered to be in remission. The average ages at onset of focal dystonia and segmental dystonia were greater than for generalized dystonia (p<0.0003). The severity of the abnormal head-neck movements were more severe among the patients with generalized dystonia (p<0.001). Pain in the cervical area was noted in 59 patients. It was not possible to determine the etiology of the disease in 62.3% of patients. Tardive dystonia was the most common secondary etiology. A major improvement in the motor symptoms of CD and pain was observed in patients following treatment with BoNT/A. The tardive dystonia subgroup did not respond to the treatment. Dysphagia was observed in 2.35% of the patients.

Huntington's disease-like 2 in Brazil--report of 4 patients.

Huntingtons disease‐like 2 (HDL2) is a neurodegenerative disorder found in people of African ancestry with clinical, radiological, and neuropathological manifestations similar to Huntingtons disease (HD). HDL2 is caused by a pathological expansion of CAG/CTG triplets in exon 2A of the JPH3 gene. We describe four cases of HDL2 from four unrelated families, and discuss their clinical findings. HDL2 should be considered in every patient with an HD‐like phenotype who tests negative for the HD mutation, even if African ancestry is not immediately apparent.

Jaw-opening oromandibular dystonia secondary to Wilson's Disease treated with botulinum toxin type A

We have reported a case series of five patients with jaw-opening oromandibular dystonia secondary to Wilsons disease (WD), in which the patients were treated with botulinum toxin type A (BTX-A). In all cases, dystonia score was partially reduced three weeks after injections. The most common side effect was transient mild dysphagia. This preliminary study showed that jaw-opening oromandibular dystonia in WD may be partially responsive to the use of BTX-A.

Cross-cultural influences on psychogenic movement disorders - a comparative review with a Brazilian series of 83 cases.

OBJECTIVE To describe the profile and presentation of a Brazilian series of psychogenic movement disorders (PMD) patients and to perform a comparative analysis with the previous series published worldwide. METHODS A total of 83 patients with a clinical diagnosis of PMD were included. All patients were assessed and followed at the Movement Disorders Outpatient clinic from 2000 to 2008. Demographic, clinical, paraclinical, and treatment outcome data were collected using a standardized protocol. The Gupta & Lang criteria were used for the diagnosis of PMD. RESULTS Seventy-three patients were females (87.95%), with mean age of onset of 39 ± 5.1 years. Tremor was the most frequent PMD, occurring in 42 (50.6%) patients followed by dystonia, in 27 (32.5%) patients. Cerebellar-like ataxia was seen in 3 patients (4.16%). Psychiatric co-morbidities were diagnosed in 67 (80.7%) of the patients. Thirty-five of the 58 patients (60.3%) who were managed and followed up for six months had some degree of improvement, including 22 patients (37.9%) with complete or almost complete remission. CONCLUSION In this series of patients with PMD, tremor and dystonia were most frequent PMD. Our results overlap those of the literature regarding most demographic and clinical findings, demonstrating the trans-cultural aspect of PMD.

Botulinum toxin type A and cervical dystonia: a seven-year follow-up

Most cases of cervical dystonia (CD) are idiopathic, and focal injections of botulinum toxin A (BoNT/A) are the treatment of choice. The objective of our study was to document the effects of long-term BoNT/A treatment in idiopathic CD patients. Fifty-eight patients with idiopathic CD were recruited from March 2001 to May 2002. Twenty-eight of the subjects were available for reassessment after seven years. During this period, all had received regular treatment with BoNT/A injections. Clinical information about patients and the severity of CD (TWSTRS and VAPS) at baseline assessment (2001-2002) and follow-up (2008-2009) was compared. Significant motor improvement was detected based on TWSTRS scale scores, which were used to analyze clinical severity (19.6 ± 6.6 and 17.7 ± 4.8; p<0.05). There was no improvement in the severity of cervical pain (p=0.43). In conclusion, BoNT/A was a safe and effective long-term therapy for CD.

Hemicoréia-hemibalismo associado a granuloma criptocócico em paciente com SIDA: relato de caso

Movement disorders are not common in acquired immunodeficiency syndrome. Hemichorea-hemiballism (HC-HB) is the most common of them all, and it is usually related to oportunistic toxoplasmosis of the basal ganglia. We present a 28-year-old man, HIV positive with HC-HB caused by a right subthalamic granuloma, which did not respond to treatment for toxoplasmosis. Cryptoccococic antigen was positive in the cerebrospinal fluid and antifungic therapy led to clinical and radiologic improvement, thus the diagnosis of a granulomatous lesion by Cryptococcus neoformans was established. Current literature on HC-HB and its relationship with AIDS is subsequently reviewed.Movement disorders are not common in acquired immunodeficiency syndrome. Hemichorea-hemiballism (HC-HB) is the most common of them all, and it is usually related to oportunistic toxoplasmosis of the basal ganglia. We present a 28-year-old man, HIV positive with HC-HB caused by a right subthalamic granuloma, which did not respond to treatment for toxoplasmosis. Cryptoccococic antigen was positive in the cerebrospinal fluid and antifungic therapy led to clinical and radiologic improvement, thus the diagnosis of a granulomatous lesion by Cryptococcus neoformans was established. Current literature on HC-HB and its relationship with AIDS is subsequently reviewed.

Cervical dystonia: about familial and sporadic cases in 88 patients

Cervical dystonia (CD) affects the musculature of the neck in a focal way or associated to other parts of the body. The aim of this study was to identify clinical differences between patients with dystonia patients without family history and with family history (sporadic). Eighty-eight patients with CD were recruited in a Movement Disorders Clinic between June of 2008 and June of 2009. Only patients with no etiological diagnosis were accepted for analysis. The age of onset of symptoms was later in patients with focal and segmental dystonia than in patients with generalized dystonia (p<0.001). The severity of symptoms was higher in patients with sporadic dystonia than in familial patients (p<0.01). Generalized cases were more severe in patients with a family history (p<0.01). Sporadic patients had higher levels of pain than familial cases (p<0.05). We expect soon to present the results of genetic analyzes of these patients.

Américo Negrette and Huntington's disease

The authors present a historical review of the seminal clinical contribution of Professor Américo Negrette, a Venezuelan neurologist, to the evolution of scientific knowledge about Huntingtons disease.