Evaluation of sympathetic skin response in Parkinson's disease.

There is no clear definition on the role of sympathetic skin response (SSR) in the evaluation of patients with Parkinson's disease (PD). We recorded the SSR of the palms of 64 controls and 46 patients with PD to electrical stimulation of the median nerve at the wrist. We analyzed onset latency and peak-to-peak amplitude. A study of parasympathetic function (R-R interval analysis) was also undertaken. We found that patients with PD had more absent SSRs than controls. The mean amplitude of the SSR was significantly reduced in both lower and upper limbs of PD patients in comparison with control subjects (p<0.001). The onset latency was longer in the lower limbs of these patients in respect to the control group (p<0.003). There was a significant inverse correlation between SSR amplitudes and age, severity and late onset of the disease. There was no association of these parameters with dysautonomic symptoms or R-R interval variation. In conclusion, there is a significant association between altered SSR and PD and an inverse correlation in this group of patients between SSR values and older age, greater severity and later onset of disease. Therefore, the study of SSR may provide valuable information on cholinergic sympathetic function in patients with PD.

Hyperhidrosis in Parkinson's disease.

We studied the sudomotor skin response (SSR) in patients with Parkinson's disease with and without symptomatic hyperhidrosis. The study was carried out in 13 patients who complained of excessive sweating and in 37 patients who did not have excessive sweating. Patients were matched for age, sex, degree of impairment, duration of the disease, and number and severity of autonomic disturbances. Excessive sweating involved mainly the face, head, and trunk. The SSR was recorded from the palm of the hands to electrical stimulation of the median nerve at the wrist. We analyzed onset latency, peak to peak amplitude, and waveform. Patients with hyperhidrosis had more often absent responses (chi(2) = 5.292; P = 0.021), their responses were of lower mean amplitude (analysis of variance [ANOVA]; F[2,101] = 11.678; P < 0.001), and they had a reduced number of responses with a predominantly negative component (chi(2) = 8.493; P = 0.004) than patients who did not complain of sweating disturbances. Our results indicate that excessive sweating in Parkinson's disease concurs with decreased activation of sweat glands in the palms of the hands and suggests that axial hyperhidrosis could be a compensatory phenomenon for reduced sympathetic function in the extremities.

Prediction of the neurophysiological diagnosis of carpal tunnel syndrome from the demographic and clinical data.

OBJECTIVE: To use demographic and clinical data to identify the clinical pattern that best predicts the diagnosis of carpal tunnel syndrome (CTS), as defined by neurophysiologic studies. METHODS: A diagnostic cross-sectional study in 2535 consecutive patients (3907 upper limbs) older than 12 years old who were referred for nerve conduction studies in the upper limbs between August 2001 and January 2003 in 3 university hospitals and 2 private neurophysiology services in the state of Rio Grande do Sul, Brazil. RESULTS: A neurophysiologic diagnosis of CTS was established in 39.1% of these upper limbs. The presence of paresthesias or pain at least 2 of the first 4 digits in association with one of the following: female gender, symptoms worsening at night or on awakening, an BMI > or =30, thenar atrophy, or other sign (Tinel's, Phalen's, or Reversed Phalen's signs); were the best pattern associated with the diagnosis. CONCLUSIONS: We have found that the clinical picture alone does not seem sufficient, in majority of the population, to correctly predict the diagnosis of CTS, as defined by median nerve neuropathy at the carpal tunnel. We believe that a compressive lesion of the median nerve at the carpal tunnel can be present both in patients with no typical symptoms of CTS (including asymptomatic individuals) and in patients in which neurophysiologic studies are negative. SIGNIFICANCE: Further studies separating patients into these groups will allow us to identify the long-term prognosis as well as the ideal therapeutic approach for each of these clinical situations.

Geradores do P50: estudo com pacientes epilépticos, submetidos á amígdala-hipocampectomia / P50 generators: study with epileptic patients submitted on the amigadala-hippocampectomy

Estudar o envolvimento do hipocampo na gênese dopotencial evocado P50 e da supressão do mesmo em pacientes com epilepsia submetidos a tratamento cirúrgico. Foi realizado um estudo transversal com grupo de controle de comparação. Excluíram-se indivíduos com outra patologia neurológica ou psiquiátrica, hipoacusia auditiva, história familiar de transtornos psicóticos, uso de antipsicóticos atípicos ou de drogas ilícitas no último mês. Os indivíduos foram separados em dois grupos: grupo 1 (n=34) - amígdala-hipocampectomia; grupo 2 (n=40) - controles. Foi realizado, em todos os participantes, o potencial evocado auditivo de m,édia latência P50. Consideraram-se variáveis de comparação

What symptoms are truly caused by median nerve compression in carpal tunnel syndrome?

OBJECTIVE: This study aims to identify the symptoms, signs and distributions which are associated with neurophysiological carpal tunnel syndrome (N-CTS), defined by the finding of a median nerve lesion at the wrist through neurophysiologic studies, and to compare them with those of patients with sensory or motor complaints in the upper limbs whose electrophysiologic investigation did not show evidence of this syndrome. METHODS: A cross-sectional study was performed, with prospective gathering of data, following a predetermined protocol. We included all patients older than 12 years who were referred for nerve conduction studies and electromyography of at least one of the upper limbs between August 2001 and January 2003. The patients answered a clinicoepidemiologic questionnaire and painted the areas in which they felt pain and those in which they felt paresthesia, and were also examined to test for classical signs CTS. RESULTS: A neurophysiological diagnosis of CTS was reached in 1549 upper limbs (39%). Approximately 6% of the upper limbs with N-CTS and 16% of those without median nerve lesion do not have any hand or wrist symptoms (P<0.001). Paresthesia, pain, hand weakness and cramps were the symptoms statistically associated to N-CTS, with paresthesia being the one with the greatest power of association. This is usually felt in median innervation's territory, and is frequently extended to the whole hand, but without association with N-CTS when felt in proximal region. CONCLUSIONS: One concludes that the most characteristic manifestation of carpal tunnel syndrome (CTS) is paresthesia. Pain also occurs very frequently but it is less specific, and weakness, meanwhile, seems to have a low frequency. We believe that the variability in the clinical presentation of CTS is largely due to the presence of associated diseases and our results provide information which could help to better define the clinical criteria used in the diagnosis of this syndrome.

Clinical features of 1039 patients with neurophysiological diagnosis of carpal tunnel syndrome.

PURPOSE: To describe the clinical features of patients with a neurophysiologic diagnosis of carpal tunnel syndrome (CTS) in the state of Rio Grande do Sul, Brazil. METHODS: We prospectively studied 1039 patients with a neurophysiologic diagnosis of CTS in southern Brazil. All patients completed a clinicoepidemiological questionnaire which included a drawing of the arm and hand, in which they were asked to paint the areas in which paresthesia and pain occurred. In part of the sample, we also investigated the presence of clinical signs such as Tinel's, Phalen, and tenar atrophy. The diagnosis of CTS was performed using a predetermined neurophysiological protocol. RESULTS: A total of 1528 hands were diagnosed with CTS. The severity of CTS was mild in 42% of cases, moderate in 18% and severe in 40%. Patients had a mean age of 48.3 +/- 12.4 years old, and a ratio of 5.6 females to 1 male was observed. Symptoms restricted to the hand and wrist was observed in 51.8% of cases with paresthesia and in 18.5% of cases with pain. In 92.5% of the partially affected hands, paresthesia was present in at least one of the first three fingers, while pain affected the three first fingers in 78.8% of these hands. Pain with a distribution which did not involve the hand occurred in 18.5% of cases, while paresthesia without involvement of the hand occurred in only 1.9%. Distribution of symptoms, according to Katz's hand diagram, showed a classic pattern in 12.6% of affected hands, a pattern classified as probable CTS in 66.3%, and an unlikely CTS pattern in 4.1%. Tinel's and Phalen's sign were observed in 34.2 and 56.3% of the hands, respectively. CONCLUSION: The clinical presentation of CTS is pleomorphic, ranging from the absence of symptoms to very severe cases. This variation probably is dependent on coexistent diseases, such as tendinitis and fibromyalgia, as well as on subjective aspects of the patients.

Seasonal distribution and demographical characteristics of carpal tunnel syndrome in 1039 patients

OBJETIVO: Descrever as características demográficas de gênero, idade e presença de movimentos repetitivos, uso de álcool e antiinflamatórios não-esteróides (AINE), especialidades médicas de encaminhamento para eletroneuromiografia (ENMG), nível de escolaridade, e distribuição sazonal em pacientes com síndrome do túnel do carpo (STC) diagnosticada neurofisiologicamente no Estado do Rio Grande do Sul, Brasil. MÉTODO: Foi estudada uma série de 1039 pacientes (1549 mãos), com STC definida neurofisiologicamente, que foram encaminhados para realização de ENMG, em 3 serviços universitários e 2 privados, entre agosto de 2001 a janeiro de 2003. Todos os pacientes responderam questionário com informações demográficas. O diagnóstico da STC foi realizado seguindo protocolo pré-estabelecido, com critérios diagnósticos definidos. RESULTADO: Cerca de um quarto dos pacientes já havia realizado ENMG prévia, sendo observada maior freqüência da STC em mulheres (5,6:1) e em pacientes acima de 40 anos. A maioria relatava esforços repetitivos na sua atividade diária (69,7%), 12,9% estava em uso de AINE e 14,9% relatavam ingesta regular de bebidas alcoólicas. Observamos maior freqüência da STC nos meses de julho e agosto em relação aos outros meses do ano. Cerca de 2/3 da nossa população apresentava pelo menos o primeiro grau completo. Na maior parte dos pedidos de ENMG não era descrita uma hipótese diagnóstica (69,9%), sendo a traumatologia/ortopedia a especialidade que mais solicitou exames neurofisiológicos (71,1%). CONCLUSAO: Concluímos que a STC, em nosso meio, apresenta características demográficas semelhantes às descritas na literatura. A maior parte de nossa amostra apresenta pelo menos o 1º. grau completo e foi encaminhada para realização de ENMG por traumatologistas. Salientamos a distribuição sazonal da STC que demonstra uma associação significativa com os meses de inverno.

Seasonal distribution and demographical characteristics of carpal tunnel syndrome in 1039 patients.

OBJECTIVE: To describe the demographic characteristics of gender, age, and presence of repetitive movements, intake of alcohol and non-steroid anti-inflammatories (NSAI), medical specialties that referred patients to nerve conduction studies and electromyography (NCS-EMG), school attainment, and seasonal distribution in patients with a neurophysiological diagnosis of carpal tunnel syndrome (CTS) in the State of Rio Grande do Sul, Brazil. METHOD: A series of 1039 patients (1549 hands) with neurophysiologically defined CTS was studied. Patients were referred for NCS-EMG in 3 universities and 2 private services, from August 2001 to January 2003. All patients completed a questionnaire containing demographic information. The diagnosis of CTS was established following a pre-established protocol, with defined diagnostic criteria. RESULTS: Around one fourth of patients had already performed NCS-EMG; the greatest frequency of CTS was observed in women (5.6:1) and in patients above the age of 40. Most patients reported performing repetitive movements in their daily routine (69.7%); 12.9% reported use of NSAI and 14.9% regular intake of alcoholic beverages. A greater frequency of CTS was observed in the months of July and August, when compared to the other months of the year. Around 2/3 of the study population had completed at least secondary school. Most requests of nerve conduction studies did not provide a diagnostic hypothesis (59.9%) and neurophysiologic studies were requested mostly by traumatology/orthopedics (71.1%). CONCLUSION: We have concluded that, in our environment, CTS shows some demographical characteristics that are similar to what the literature describes. Also, we have found that most of our sample concluded at least secondary school, and was referred to neurophysiologic studies by orthopedists. To be pointed out is the seasonal distribution of CTS, which demonstrates a significant association with winter months.

Clozapine, but not typical antipsychotics, correct P50 suppression deficit in patients with schizophrenia.

OBJECTIVE: To find out if there is a difference in P50 suppression between patients using typical antipsychotic drugs and those using clozapine, as well as to confirm the findings of abnormal P50 suppression in patients with schizophrenia, when compared to healthy volunteers. METHODS: Fifty patients with schizophrenia and 25 healthy volunteers were divided into 3 groups: group 1 - patients using typical antipsychotics; group 2 - patients using clozapine; group 3 - controls. Before the examination, all patients were interviewed by a psychiatrist using the Brief Psychiatry Rating Scale (BPRS). RESULTS: The average S2/S1 ratio was 0.82+/-0.45 in group 1, 0.57+/-0.41 in group 2, and 0.44+/-0.27 in group 3 (P=0.003). Statistical analysis showed a significant difference when the results of group 1 were compared to those of groups 2 (P=0.045) and 3 (P=0.001). There was no significant difference between groups 2 and 3 (P=0.182). There was a significant difference in the S1-S2 difference only between groups 1 and 3 (P=0.007), but a non-significant trend towards a similar difference was found between groups 1 and 2 (P=0.067). There was no correlation between the BPRS values and any P50 parameter. CONCLUSIONS: The suppression of P50 among patients using clozapine was significantly greater than that obtained in patients using typical antipsychotics. SIGNIFICANCE: This study confirms, in a more evident way, the improvement of the suppression of P50 potential in schizophrenics using clozapine. Additionally, it discusses the physiopathological mechanism involved.

Nerve conduction studies, electromyography and sympathetic skin response in Fabry's disease.

We prospectively performed neurophysiologic studies in nine Fabry's Disease (FD) patients (8 male and 1 female) in order to describe the results of nerve conduction studies (NCS) and electromyography (EMG) and to verify whether the sympathetic skin response (SSR) is impaired in these patients. The investigation protocol included SSR, sensory and motor NCS and EMG. SSR was performed not only in FD patients, but also in 18 normal controls. All FD patients had normal nerve conduction studies and electromyography. SSR was present in all controls with a mean amplitude of 1453.6+/-682.3 microV. However, the SSR was absent in six and lower than 500 microV in the remaining FD patients. All patients had normal sensory and motor NCS and EMG. SSR, on the other hand, was significantly altered in all patients and this test could, therefore, be useful in the diagnostic evaluation of FD patients.

[Progressive bulbar palsy (Fazio-Londe disease): case report]. / Paralisia bulbar progressiva juvenil doença de Fazio-Londe: relato de caso.

Progressive bulbar palsy, also called Fazio -Londe disease, is characterized by progressive impairment of cranial nerves in children. It was first reported by Fazio in 1892 and until now only 30 cases have been published in the literature. Both sexes can be affected and clinical course can be divided on early (< 6 years age, predominance of respiratory symptoms) and late course (6-20 years of age, predominance of motor symptoms on superior limbs). We report a 4 years old boy that started with intense stridor and respiratory distress, initially being diagnosed as an acute asthma attack. Clinical signs worsened and 12 months latter he already had impairment of cranial nerves V, VII, VIII, IX and X confirmed by clinical examination and neurophysiological evaluation.

Paralisia bulbar progressiva juvenil doença de Fazio-Londe: relato de caso / Progressive bulbar palsy (Fazio-Londe disease): case report

A paralisia bulbar progressiva, também denominada doença de Fazio-Londe, caracteriza-se pelo acometimento degenerativo progressivo de nervos cranianos bulbares em crianças. Foi descrita primeiramente por Fazio em 1892 e até a presente data somente 30 casos foram relatados na literatura. Acomete ambos os sexos, assumindo dois padröes clínicos, um de início precoce (idade <6 anos, predomínio de comprometimento respiratório) e outro de início tardio (6-20 anos, predomínio de comprometimento motor nos membros superiores). Descrevemos o caso de um menino com 4 anos, cujo primeiro sintoma foi estridor e disfunçäo respiratória grave, tendo inicialmente recebido diagnóstico de asma brônquica. O quadro evoluiu progressivamente, em aproximadamente 12 meses, para paralisia do V, VII, VIII, IX e X nervos cranianos, comprovada por acompanhamento clínico, exame físico e estudo eletrofisiológico de tronco cerebral