Response to heat pain stimulation in idiopathic Parkinson's disease.

BACKGROUND: Pain is a prominent nonmotor symptom in Parkinson's disease (PD) but has not been well studied. OBJECTIVE: The aim of this study is to assess thermal experience and emotional content, as well as side-to-side sensory differences in PD "off" and "on" dopaminergic therapy following thermal cutaneous stimulation. DESIGN: Cross-sectional design. SETTING: University teaching hospital. METHODS: Twelve PD subjects experiencing motor fluctuations but no pain symptoms and 13 healthy controls participated in the study. Heat pain and emotional content were assessed using a thermode and visual analog scales in medication on and off states in PD and without medication in healthy controls. RESULTS: There were no side to side differences in heat pain intensity or between PD medication on state and PD medication off state. Unexpectedly, PD subjects reported a higher degree of unpleasantness in response to heat pain while on medication compared with the off state. CONCLUSIONS: These results suggest that the perception of heat pain is mediated, at least in part, by nondopaminergic systems in PD, while dopamine might modulate the affective component of pain.

Dopamine transporter PET in normal aging: dopamine transporter decline and its possible role in preservation of motor function.

OBJECTIVES: To determine the impact of age-related decline in dopamine transporter (DAT) expression on motor function in the elderly. METHODS: About 33 normal individuals of a wide age range were scanned with PET employing d-threo-[(11)C]-methylphenidate (MP, a marker of DAT) and [(11)C]-dihydrotetrabenazine (DTBZ, that binds to the vesicular monoamine transporter Type 2). Motor function was assessed using the Purdue Pegboard Test (PPB). We analyzed the relationship between [(11)C]-MP and motor performance. RESULTS: Age ranged from 27- to 77-year old (mean +/- SD, 54.75 +/- 14.14). There was no age-related decline in binding potentials (BP) for [(11)C]-DTBZ. In contrast, [(11)C]-MP BP was inversely related to age in all striatal regions analyzed (caudate: reduction of 11.2% per decade, P < 0.0001, r = -0.86; putamen: reduction of 10.5% per decade, P < 0.0001, r = -0.80). A differential effect of [(11)C]-MP on PPB could be observed according to age group. There was a positive relation between the PPB and [(11)C]-MP in young individuals (coefficient = 13.56), whereas in individuals greater than 57 years this relationship was negative (coefficient = -19.53, P = 0.031). CONCLUSIONS: Our findings confirm prior observations of age-related DAT decline and suggest that this phenomenon is independent of changes in VMAT2. After the fifth decade of life, this reduction in DAT binding is associated with a motor performance comparable to mid-adult life. These findings imply that biochemical processes associated with healthy aging may offset the naturaldecline in motor function observed in the elderly.

Dopamine transporter relation to dopamine turnover in Parkinson's disease: a positron emission tomography study.

OBJECTIVE: To investigate the role of the dopamine transporter (DAT) in the regulation of synaptic dopamine (DA) levels in Parkinson's disease and its role in the preservation of DA in presynaptic terminals. METHODS: Ten Parkinson's disease patients (age, 62.9 +/- 9.5 years; Unified Parkinson's Disease Rating Scale motor score in "off" state, 28.5 +/- 8.2) underwent positron emission tomography with (11)C-methylphenidate (MP, a DAT marker), (11)C-dihydrotetrabenazine (a vesicular monoamine transporter 2 marker), and (18)F-fluorodopa, leading to the determination of the MP and (11)C-dihydrotetrabenazine binding potentials (BPs) and the effective distribution volume for (18)F-fluorodopa, the inverse of DA turnover. Seven patients also underwent positron emission tomography with (11)C-raclopride before and 1 hour after levodopa administration to estimate levodopa-induced changes in synaptic DA concentration. RESULTS: We found a significant positive correlation between effective distribution volume and BP(MP) (r = 0.93; p < 0.001) and a significant negative correlation between changes in synaptic DA concentration and BP(MP) (r = -0.93; p = 0.04), independent of disease severity and duration. INTERPRETATION: These data show that in Parkinson's disease, greater DAT levels are directly associated with lower DA turnover and lower changes in synaptic DA concentration. This implies that an important functional role of DAT is to maintain relatively constant synaptic DA levels and to preserve DA in nerve terminals. A decrease in DAT, although potentially serving as a compensatory mechanism in early disease, may ultimately result in increased DA turnover and higher oscillations in synaptic DA concentration, thereby possibly predisposing toward the occurrence of motor complications as disease progresses.

Bone marrow transplantation in patients with storage diseases: a developing country experience.

Bone marrow transplantation (BMT) is a therapeutic option for patients with genetic storage diseases. Between 1979 and 2002, eight patients, four females and four males (1 to 13 years old) were submitted to this procedure in our center. Six patients had mucopolysaccharidosis (MPS I in 3; MPS III in one and MPS VI in 2), one had adrenoleukodystrophy (ALD) and one had Gaucher disease. Five patients had related and three unrelated BMT donor. Three patients developed graft versus host disease (two MPS I and one MPS VI) and died between 37 and 151 days after transplantation. Five patients survived 4 to 16 years after transplantation. Three patients improved (one MPS I; one MPS VI and the Gaucher disease patient), one patient had no disease progression (ALD) and in one patient this procedure did not change the natural course of the disease (MPS III).

Movement disorders in Latin America.

The authors review some particularities of movement disorders (MDs) in the ethnically diverse population of Latin America. Although idiopathic diseases are evenly prevalent, access to treatment encounters difficulties that are worth discussing. Infectious-parasitic diseases observed throughout the continent occasionally present as MDs, and will be individually reviewed. Inherited MDs with regional foci of increased prevalence, particularly spinocerebellar ataxias, will also be considered. Whereas there is no treatment for genetic disorders, most of the other conditions are preventable or amenable to adequate treatment. Hope for better health standards for the Latin American population lies in profound social and political changes that are still to come.

Bone marrow transplantation in patients with storage diseases: a developing country experience

O transplante de medula óssea é uma opção terapêutica para os pacientes com doenças de acúmulo. Entre 1979 e 2002, oito pacientes, quatro femininos e quatro masculinos (entre um e 13 anos de idade) foram submetidos a este procedimento em nosso centro. Seis pacientes apresentavam mucopolissacaridose (MPS I em 3; MPS III em um e MPS VI em 2), um paciente apresentava adrenoleucodistrofia e um apresentava doença de Gaucher. Cinco pacientes receberam o transplante de doador aparentado e três de doador não aparentado. Três pacientes desenvolveram doença do enxerto versus hospedeiro (dois com MPS I e um com MPS VI) e faleceram entre 37 e 151 dias após o transplante. Cinco pacientes sobreviveram entre 4 e 16 anos após o transplante. Três tiveram melhora clínica (um MPS I, um MPS VI e o paciente com doença de Gaucher), um paciente não apresentou progressão da doença (adrenoleucodistrofia) e um paciente não teve alteração da história natural da doença (MPS III).

Parkinson's disease: in vivo assessment of disease progression using positron emission tomography.

Over the past two decades, positron emission tomography (PET) has provided valuable insights into the mechanisms of nigrostriatal degeneration in Parkinson's disease (PD). Furthermore, it allows the in vivo assessment of disease progression and the evaluation of treatment interventions. In this review, we shall discuss some of the issues and concerns that arise with the use of PET as a surrogate marker of disease progression in Parkinson's disease.

Parkinsonism associated with neurocysticercosis.

We report two cases of brainstem cysticercotic ependymitis presenting with rigid-akinetic syndrome. There was a good response to levodopa therapy as well as cysticidal therapy with albendazole, allowing later reduction of levodopa dosage in one patient and complete withdrawal in the other.

Cerebellar hemorrhage as a complication of temporal lobectomy for refractory medial temporal epilepsy: report of three cases.

Cerebellar hemorrhage is listed among the potential complications following neurosurgical procedures. In this scenario it is usually reported as a rare condition. However, it seems that epilepsy surgery patients are somewhat more prone to this kind of complication, compared to other surgical groups. Head positioning, excessive cerebral spinal fluid draining and the excision of non-expanding encephalic tissue (or combinations among the three) are likely to be cause underlying remote cerebellar hemorrhage. Out of the 118 ATL/AH performed at our institution, between 1996 and 2002, we identified 3 (2.5%) patients presenting with cerebellar hemorrhage. We report on such cases and review the literature on the topic.

Carotid transient ischemic attacks presenting as limb-shaking syndrome: report of two cases.

Limb shaking syndrome (LSS) is a rare presentation of transient ischemic attacks (TIAs), usually secondary to a critical carotid stenosis compromising intracranial circulation, first described 40 years ago. Two additional cases are described herein, aiming to add on to previous descriptions, and to warn physicians about this potentially harming and rather uncommon condition.

Parkinson's disease and thyroid dysfunction.

BACKGROUND: Although no causal linkage between hypothyroidism and Parkinson's disease (PD) has been demonstrated so far, both share common manifestations and coexistence can be a source of diagnostic delay and confusion. PURPOSE: To assess thyroid function in patients with PD. SCOPE: Ninety-five PD patients and 102 age-matched controls were assessed for hypothyroidism through clinical evaluation and laboratory screening. RESULTS: In the PD group 13.7% were hypothyroid versus 10.8% in the control group. Statistical analysis did not show a significant difference (p = 0.3681). CONCLUSION: Although hypothyroidism was not more prevalent in our PD patients in comparison with the control group, we believe that given the potential overlap of symptoms and signs, thyroid function should be assessed in patients showing worsening of symptoms that cannot be explained by disease progression or resistance to therapy adjustment; screening should take into account the particular acute effect of levodopa on serum TSH levels.

Carotid transient ischemic attacks presenting as limb-shaking syndrome: report of two cases

A síndrome do membro tremulante, inicialmente descrita há 40 anos, é apresentação rara de ataques isquêmicos transitórios, sendo usualmente secundária a estenose carotídea crítica comprometendo a circulação intracraniana. Dois novos casos são aqui descritos, visando ilustrar e discutir esta condição incomum e com alto potencial de morbidade.

Cerebellar hemorrhage as a complication of temporal lobectomy for refractory medial temporal epilepsy: report of three cases

A hemorragia cerebelar faz parte das potenciais complicações dos procedimentos neurocirúrgicos. De forma geral, é considerada uma condição rara. Entretanto, há aparente propensão dos pacientes sub metidos ao tratamento cirúrgico de epilepsia em apresentar este tipo de complicação, quando compara dos com outros grupos cirúrgicos. O posicionamento da cabeça, excessiva drenagem de líquido cefalorraquidiano e a excisão de tecido cerebral não expansível (ou talvez combinações entre os três) constituem as po ten ciais causas da hemorragia cerebelar remota. Entre os 118 pacientes em nossa série de LTA » AH, identifi camos 3(2.5%) casos de hemorragia cerebelar. Relatamos os três casos desta natureza, com revisão da lite ratura pertinente a esta complicação.

[Clinical response to long action propranolol in 40 patients diagnosed with essential tremor with no previous treatment: an open, non-controlled study]. / Uso do propranolol de ação prolongada em 40 pacientes com tremor essencial e virgens de tratamento: um ensaio clínico não controlado.

UNLABELLED: Essential tremor (ET) is the most common movement disorder and betablockers are still considered the first line of treatment. The aim of our study is to report the clinical response to long action propranolol (LAP) of 40 patients diagnosed with essential tremor with no previous treatment. METHOD: 40 patients with ET were evaluated with rating scales for severity of tremor and clinical classification of ET. All patients were evaluated at least twice, at enrollment and one month after starting treatment. RESULTS: thirty-six patients (90%) had type 2 ET and types 3 and 4 occurred in two patients each (10%). Familiar history was positive in 25 patients (62.5%). Mean age at first evaluation was 43.1 years and mean age at onset was 27.4 years. Of all patients, 33 (82.5%) had some degree of benefit and in 52.5 % this benefit was either good or excellent. CONCLUSION: LAP seems to be a good treatment option for ET in our series of 40 patients.

Flunarizine and cinnarizine-induced parkinsonism: a historical and clinical analysis.

BACKGROUND: Drug-Induced Parkinsonism (DIP) represents the second leading cause of Parkinsonism (PK) in several countries. Flunarizine and cinnarizine are some of the most common drugs that cause DIP. This paper reviews the first description of Flunarizine and Cinnarizine-Induced Parkinsonism (FCIP), as well as the subsequent literature, emphasizing epidemiological, clinical and diagnostic aspects. METHODS: We reviewed the literature on the subject, with special emphasis on the first description and the later definition of the clinical syndrome that results from chronic use of flunarizine and cinnarizine. RESULTS: In 1984, De Melo-Souza reported the first description of flunarizine-induced PK in five patients. Other reports followed on FCIP, emphasizing the clinical features, which are symmetrical parkinsonism, and depression, affecting mainly elderly women. CONCLUSIONS: Eighteen years after the original description, FCIP is a recognized condition with specific clinical features, and is the second most common cause of parkinsonism in many countries.

Uso do propranolol de ação prolongada em 40 pacientes com tremor essencial e virgens de tratamento: um ensaio clínico não controlado / Clinical response to long action propranolol in 40 patients diagnosed with essential tremor with no previous treatment: an open, non-controlled study

O tremor essencial (TE) é o distúrbio do movimento mais frequente. Entre os tratamentos de primeira escolha está o uso de beta-bloqueadores. O objetivo deste trabalho é relatar os resultados do uso de propranolol de ação prolongada (PAP) em 40 pacientes com TE e virgens de tratamentos anteriores. MÉTODO: 40 pacientes com TE foram submetidos a um protocolo de avaliação pré-estabelecido em que constavam escalas de classificação para o tremor e escalas de avaliação da severidade do tremor. Todos os pacientes foram submetidos a avaliação inicial e após 1 mês de tratamento. RESULTADOS: com relação ao tipo de tremor, 36 pacientes (90 por cento do total) tinham o tipo 2; os tipos 3 e 4 ocorreram em dois pacientes cada (10 por cento do total). Houve história familiar de tremor em 25 casos (62,5 por cento). A média de idade dos pacientes foi 43,1 anos e a média de idade de início dos sintomas foi 27,4 anos. Dos 40 indivíduos avaliados, 33 ou 82,5 por cento apresentaram algum grau de melhora com PAP; em 52,5 por cento a melhora foi considerada ótima ou boa. CONCLUSAO: o PAP mostrou ser uma medicação adequada para o tratamento do TE nesta amostra de 40 pacientes avaliados.

Botulinum toxin for treatment of Frey's syndrome: report of two cases.

Frey's syndrome is a phenomenon of hemifacial flushing and sweating after gustatory stimulus, usually secondary to surgical trauma over the parotid gland, although other injury mechanisms may be seen. It is accepted as a result of aberrant regeneration of facial autonomic nerve fibers. Treatment evolved from ineffective medical and surgical approaches to botulinum toxin. We evaluate the effectiveness and safety of botulinum toxin in the treatment of this complication in two patients.

Botulinum toxin for treatment of Frey's syndrome: report of two cases

Frey's syndrome is a phenomenon of hemifacial flushing and sweating after gustatory stimulus, usually secondary to surgical trauma over the parotid gland, although other injury mechanisms may be seen. It is accepted as a result of aberrant regeneration of facial autonomic nerve fibers. Treatment evolved from ineffective medical and surgical approaches to botulinum toxin. We evaluate the effectiveness and safety of botulinum toxin in the treatment of this complication in two patients