Dermatoses in parkinsonism: the importance of multidisciplinary follow-up

Summary Parkinsonism is characterized by bradykinesia with rigidity and/or resting tremor, in addition to non-motor symptoms, which include dermatological manifestations. The objective of this study is to evaluate the main dermatoses in patients with parkinsonism found at the Philanthropic Association of Curitiba - PR. A cross-sectional descriptive study was carried out with the application of a questionnaire and dermatological evaluation of the patients. The sample consisted of 386 patients and was composed mainly by men (55.4%), between 60-74 years old (51.6%), with complete primary education (45.3%), disease diagnosis time between 5-10 years (35%) and in use of medication (96.6%). The most prevalent dermatoses were pigmented nevus (36.3%), warts (25.1%), actinic keratosis (22%), seborrheic keratosis (21.5%), seborrheic dermatitis (20.5%), and rosacea (19.2%). Among the 13 cases (3.4%) of malignant cutaneous neoplasms confirmed by biopsy, 2 were melanomas. Regarding patients' sex, there was a higher prevalence of inflammatory dermatoses (OR 1.64, 95% CI 1.08-2.51, p = 0.025) and benign cutaneous neoplasms (OR 1.77, 95% CI 1.16-2.69, p = 0.01) in men. As to age, patients aged between 60-74 years had more pre-malignant skin lesions (OR 2.60, 95% CI 1.05-6.44, p <0.001) and seborrheic keratosis (OR 2.52, 95% CI 1.02-6.25, p = 0.001) and, in those older than 75 years, actinic keratosis was more frequent (OR 5.43, 95% CI 2.17-13.6, p <0.001). The results of the study show that it is fundamental to dermatologically evaluate and monitor these patients, aiming at diagnosis and early treatment of lesions, especially of skin cancer.

RESUMO Parkinsonismo é caracterizado por bradicinesia e/ou tremor de repouso, além de sintomas não motores, entre os quais se destacam as manifestações dermatológicas. O objetivo desse trabalho é conhecer as principais dermatoses em pacientes portadores de parkinsonismo atendidos em uma associação filantrópica de Curitiba/PR. Foi realizado um estudo descritivo transversal com aplicação de questionário e avaliação dermatológica dos pacientes. A amostra estudada consistiu de 386 pacientes e foi composta principalmente por homens brancos (55,4%), entre 60-74 anos (51,6%), ensino fundamental completo (45,3%), tempo de diagnóstico da doença entre 5-10 anos (35%) e em uso de medicação (96,6%). As dermatoses mais encontradas na inspeção dermatológica foram manchas pigmentadas (36,3%), verrugas (25,1%), ceratose actínica (22%), ceratose seborreica (21,5%), dermatite seborreica (20,5%) e rosácea (19,2%). Entre os 11 casos (2,8%) de neoplasias cutâneas malignas confirmados por biópsia, dois eram melanomas. Em relação ao sexo, houve prevalência em homens de dermatoses inflamatórias (OR 1,64, IC 95% 1,08-2,51; p=0,025) e neoplasias cutâneas benignas (OR 1,77, IC 95% 1,16-2,69; p=0,01). Quanto à idade, pacientes entre 60-74 anos apresentaram mais lesões cutâneas pré-malignas (OR 2,60, IC 95% 1,05-6,44; p<0,001) e a ceratose seborreica (OR 2,52, IC 95% 1,02-6,25; p=0,001); naqueles acima de 75 anos foi mais frequente a ceratose actínica (OR 5,43, IC 95% 2,17-13,6; p<0,001). Os resultados encontrados no estudo evidenciam que são fundamentais a avaliação e o monitoramento dermatológico desses pacientes, visando diagnóstico e tratamento precoce das lesões, em especial do câncer de pele.

TeleCondutas: tremor e síndromes parkinsonianas / TeleGuides: tremor and parkinsonian syndromes

As principais causas de tremor em pacientes atendidos na Atenção Primária à Saúde são: exacerbação de tremor fisiológico, tremor essencial (acomete 5% da população acima de 40 anos) e as síndromes parkinsonianas. É importante definir corretamente sua origem, pois o tratamento e o prognóstico são variados. Esta guia apresenta informação que orienta a conduta para casos de tremor e síndromes parkinsonianas no contexto da Atenção Primária à Saúde, incluindo: etiologia do tremor e síndromes parkinsonianas, avaliação clínica, tipos de tremor, sintomas cardinais de parkinsonismo, medicamentos indutores, fluxograma de avaliação do tremor, exames complementares, tratamento do tremor essencial e doença de parkinson, encaminhamento para serviço especializado.

Síndrome de opsoclonia-mioclonia-ataxia em paciente com AIDS / Opsoclonus-myoclonus-ataxia syndrome in an AIDS patient

É relatado aqui o caso de uma mulher de 38 anos com AIDS que desenvolveu a síndrome de opsoclonia-mioclonia-ataxia em um período diferente dos outros casos já relatados na literatura. A síndrome de opsoclonia-mioclonia-ataxia já tinha sido relatada como manifestação inicial de AIDS, assim como no momento da soroconversão de HIV e na síndrome de reconstituição imune. Este caso é único, uma vez que a paciente tinha contagem elevada de CD4 e carga viral negativa no momento em que a síndrome de opsoclonia-mioclonia-ataxia ocorreu.

We report the case of a 38-year-old woman with AIDS who developed opsoclonus-myoclonus-ataxia syndrome during a period different from other cases reported in literature. Opsoclonus-myoclonus-ataxia syndrome had already been reported as the initial neurological presentation of AIDS, as well as at the time of HIV-seroconversion and immune reconstitution syndrome. Our case is unique since the patient had an elevated CD4 count and negative viral load in the period when the opsoclonus-myoclonus-ataxia syndrome occurred.

Desordens do movimento: 40 aspectos e muitas dicas - Neurossemiologia / Movement disorders: 40 aspects and many tips – Neurosemiology

Os autores ressaltam aspectos neurossemiológicos que contribuem para a caracterização fenotípica de diversas desordens do movimento. Devido à alta prevalência, atenção maior é dada à doença de Parkinson e às síndromes parkinsonianas, sem contudo desprestigiar os tremores e outras hipercinesias tais como: atetose, balismo, coreia, distonias, mioclonias e as desordens do movimento de origem psicogênica...

The authors point out neurosemiologic aspects that contribute to phenotypic characterization of several movement disorders. Due to high prevalence, the authors mainly address Parkinson's disease and parkinsonian syndromes, but also consider tremors and other hyperkinesias such as: athetosis, ballism, chorea, dystonia, myoclonus and movement disorders of psychogenic origin...

Espectro das anormalidades óculo-palpebrais na paralisa supranuclear progressiva (PSP) ao exame à beira-do-leito / The ocular-eyelid spectrum abnormalities in progressive supranuclear palsy (PSP) in the bedside examination

As alterações da motilidade ocular compõem um dos pilares do complexo sintomático da Paralisia Supranuclear Progressiva. Alguns sinais, tanto oculares quanto palpebrais, podem ser facilmente evidenciados ao exame neuro-oftalmológico à beira-do-leito. Neste artigo os autores ressaltam alguns aspectos óculo-palpebrais que auxiliam no diagnóstico desta curiosa e incomum síndrome parkinsoniana tau-positiva.

The alterations of ocular motility make up one of the pillars of the Progressive Supranuclear Palsy symptom complex. Some signs, both ocular and of the eyelids, can easily be shown during a neuro-ophthalmologic bedside examination. In this article the authors highlight some aspects of motor ocular and eyelid alterations to help diagnose this curious and unusual tau-positive parkinsonian syndrome.

Neuroimaging in Parkinsonism: a study with magnetic resonance and spectroscopy as tools in the differential diagnosis / Neuroimagem no parkinsonismo: estudo com ressonância magnética e espectroscopia por ressonância como ferramentas no diagnóstico diferencial

The differential diagnosis of Parkinsonism based on clinical features, sometimes may be difficult. Diagnostic tests in these cases might be useful, especially magnetic resonance imaging, a noninvasive exam, not as expensive as positron emission tomography, and provides a good basis for anatomical analysis. The magnetic resonance spectroscopy analyzes cerebral metabolism, yielding inconsistent results in parkinsonian disorders. We selected 40 individuals for magnetic resonance imaging and spectroscopy analysis, 12 with Parkinson's disease, 11 with progressive supranuclear palsy, 7 with multiple system atrophy (parkinsonian type), and 10 individuals without any psychiatric or neurological disorders (controls). Clinical scales included Hoenh and Yahr, unified Parkinson's disease rating scale and mini mental status examination. The results showed that patients with Parkinson's disease and controls presented the same aspects on neuroimaging, with few or absence of abnormalities, and supranuclear progressive palsy and multiple system atrophy showed abnormalities, some of which statistically significant. Thus, magnetic resonance imaging and spectroscopy could be useful as a tool in differential diagnosis of Parkinsonism.

O diagnóstico diferencial do parkinsonismo baseado em parâmetros clínicos pode ser difícil. Alguns exames complementares podem ser úteis, especialmente a ressonância magnética, um método não invasivo, de menor custo quando comparado a tomografia por emissão de pósitrons, proporcionando uma análise anatômica satisfatória. A ressonância por espectroscopia analisa o metabolismo cerebral, com resultados variáveis na literatura no estudo das síndromes parkinsonianas. Selecionamos 40 indivíduos para realização de ressonância magnética e espectroscopia, sendo 12 com doença de Parkinson, 11 com paralisia supranuclear progressiva, 7 com atrofia de múltiplos sistemas tipo parkinsoniana e 10 indivíduos sem manifestações neurológicas ou psiquiátricas (grupo controle). As escalas clínicas analisadas foram a de Hoenh e Yahr, unified Parkinson's disease rating scale e o mini-exame do estado mental. Os resultados encontrados revelaram que pacientes com doença de Parkinson e controle apresentavam em geral o mesmo aspecto por imagem enquanto os grupos paralisia supranuclear progressiva e atrofia de múltiplos sistemas com anormalidades, havendo significância estatística em algumas variáveis. A ressonância magnética e a espectroscopia podem ser úteis no diagnóstico diferencial do parkinsonismo.

Diagnóstico premotor de la enfermedad de Parkinson / Premotor diagnosis of Parkinson's disease

Parkinson is a neurodegenerative disease that compromises brain nuclei in an ordered pattern. Only when Sustancia Nigra compromise becomes severe, motor signs are noticed. This occurs late in Parkinson course, so there is a long time period before in which no motor signs are evident. This is “premotor phase” of Parkinson disease. To make diagnosis early in premotor phase, would allow beginning a potential treatment that modify disease course. In this article we discuss clinical picture of premotor phase of Parkinson disease, local reality about ancillary tests to improve diagnosis sensibility of it and we suggest treatment approach.

Correlações anatomoclínicas na doença de Parkinson / Anatomoclinical Correlation in Parkinson´s Disease

Braak et al., em 2003, sugeriram que o processo patológico da doença de Parkinson se iniciava nos núcleos motor dorsal do vago e olfativo anterior, com progressão caudo-rostral, em seis estágios. O presente trabalho tem por finalidade correlacionar esses estágios neuropatológicos com as suas respectivas manifestações clínicas. No estágio 1, as alterações bulbares e no núcleo olfativo anterior levam a constipação intestinal, a distúrbios do sono e a hiposmia. No estágio 2, o comprometimento pontino pode induzir a depressão, a ansiedade, a distúrbios do sono e a dor de origem central. No estágio 3, a degeneração no mesencéfalo determina o aparecimento dos sintomas motores clássicos, dos distúrbios cognitivos leves e do ciclo sono-vigília. No estágio 4, as lesões saem do tronco cerebral e atingem, principalmente, o mesocórtex temporal e a amígdala, gerando as disfunções mnemônicas, executivas e a apatia. No estágio 5, as alterações acometem o neocórtex com destaque para as áreas pré-frontais e de associação sensitivas, acentuando as disfunções cognitivas. No estágio 6, a etapa mais avançada, ocorre o comprometimento difuso das áreas corticais primárias e, por conseqüência, o agravamento das dificuldades motoras e do quadro demencial. Os Autores consideram que essas observações possam contribuir, no futuro, para possíveis benefícios terapêuticos.

Braak et al., in 2003, suggested that the pathological process of Parkinson?s disease begins at the dorsal motor nucleus of the vagus and the anterior olfactory nucleus, in a six-stage caudorostral progression. The objective of our study is to correlate these neuropathological stages with their clinical manifestations. Stage 1-related pathology started at the medulla oblongata and the anterior olfactory nucleus, leading to intestinal constipation, sleep disorders and hyposmia. At stage 2 the lesions on the pons produce symptoms of depression, anxiety, sleep disorders and central pain. In the third stage the degeneration reaches the midbrain and the classical motor symptoms appear as well as mild cognitive and sleep disorders. Stage 4 - related lesions advance upwards and exit the brain stem to the temporal mesocortex and amygdala resulting in mnemonic and executive dysfunctions and apathy. At stage 5 the lesions progress to the neocortex, mainly the prefrontal and sensory association areas, worsening the cognitive dysfunctions. At stage 6, the most advanced phase, there is the spreading of the process into the primary cortical areas, further worsening the motor symptoms and dementia. The Authors speculate that these observations could contribute to the improvement of therapeutic strategies in the future.

High occurrence and low recognition of Parkinsonism (and possible PD) in old age homes in Bangalore, South India.

BACKGROUND: The elderly population in developing countries is likely to increase by 200-280%. Age related diseases like Parkinsonism are also likely to increase in ageing population. The prevalence and awareness of Parkinsonism (and possible PD) amongst them are unknown. METHODS AND MATERIAL: The objective was to know the awareness and occurrence of Parkinsonism (and possible PD) in Old Age Homes in Bangalore, South India. The study design was prospective, direct clinical evaluation, and it was old age homes in Bangalore, South India setting. There were six hundred and twelve residents of the old age homes in Bangalore. A movement disorder neurologist examined 612 elderly residents living in Old age Homes in Bangalore city, India. RESULTS: Parkinsonism was diagnosed in 109 (17.8%) of 612 residents. Possible PD was diagnosed in 9 (1.5% of 612) while in 100 (16.3% of 612) definite PD was diagnosed.94 (86.2%) had bilateral Parkinsonian signs (Stage > or = 2 of Hoehn & Yahr), only 4 (3.7%) of them or the caregivers knew they had PD. CONCLUSIONS: Knowledge about the disease was very low in the elderly residents although the occurrence of Parkinsonism was very high. Improving awareness of PD amongst the elderly and their caregivers might reduce their disability and improve their quality of life.

Non-choreic movement disorders as initial manifestations of Huntington's disease

We describe seven patients with genetically confirmed Huntington's disease (HD) who had non-choreic movement disorders as presenting symptoms or signs. Patients with movement disorders other than chorea in the early stages tended to have larger CAG trinucleotide repeat expansion in comparison with more "typical" HD patients.

Nós descrevemos sete pacientes com doença de Huntington, geneticamente confirmada, cuja apresentação motora inicial foi diferente de coréia. Pacientes com manifestação motora inicial diferente de coréia apresentaram maior número de expansões repetidas de CAG trinucleotídeo quando comparados com aqueles com sintomatologia motora "típica".

Parkinsonism and tremor disorders: a clinical approach

Differentiation of idiopathic Parkinson's disease from other causes of Parkinsonism, such as Multiple System Atrophy, Progressive Supranuclar Palsy and Vascular Parkinsonism can be difficult. Clinicopathological studies suggest that the clinical diagnosis of idiopathic Parkinson's disease is 76% reliable. Also, clinical differentiation of tremor prominent Parkinsonism from Essential Tremor or Drug induced Parkinsonism may be problematic, especially in the early stages of the disease. Since these disorders are obviously different in clinical progress, it is important for the clinician to address the patient's and family's concerns about prognosis from a firm diagnostic footing. In this article the clinical features of the common and important causes of Parkinsonism and tremor disorders are reviewed and a practical approach is suggested

Encephalitis lethargica like illness: case report and literature review.

Recent studies have revealed that encephalitis lethargica (EL) may not be related to influenza virus infection but more likely to be a post-infectious autoimmune disease. The diagnostic clinical criteria for EL like illness include subacute hypersomnolence and ophthalmoparesis followed by Parkinsonism, oculogyric crisis, neuropsychiatric disorders and central respiratory abnormality. Recently, Magnetic Resonance Imaging (MRI), which depicts hypersignal intensity on T2 weighted, and FLAIR images at midbrain, tegmentum, and basal ganglia, have been very helpful diagnostic tests in EL like illness. Nevertheless, EL like illness has never been reported in Thailand. A 17 year-old man presented with hypersomnolence one week before admission. Physical examination revealed drowsiness and ophthalmoparesis. MRI showed bilateral hypersignal intensity lesions on T2 weighted and FLAIR images at midbrain, basal ganglia and temporal lobes. CSF studies showed normal profiles. CSF-PCR for herpes simplex virus, varicella zoster virus, cytomegalovirus, Epstein-Barr virus, Pan-Enterovirus and Westnile virus were negative. CSF Dengue and Japanese encephalitis virus hemagglutination test were negative. He was treated with intravenous dexamethasone and immunoglobulin. Somnolence and ophthalmoparesis were improved. Two months later, he developed schizophreniform features and Parkinsonism. MRI revealed improvement of midbrain and basal ganglia lesions. CSF studies showed normal CSF profiles while oligoclonal bands were positive. Autoimmune profiles and serological tests for post-streptococcal infection as well as syphilis were negative. Thyroid function tests and serum ceruloplasmin were within normal limits. Levo-Dopa, clonazepam and sodium valproate had been prescribed and the clinical syndrome was gradually improved.

Prion disease resembling frontotemporal dementia and parkinsonism linked to chromosome 17

OBJECTIVE: To compare the clinical features of a familial prion disease with those of frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17). BACKGROUND: Prion diseases are not usually considered in the differential diagnosis of FTDP-17, since familial Creutzfeldt-Jakob disease (CJD), the most common inherited prion disease, often manifests as a rapidly progressive dementia. Conversely, FTDP-17 usually has an insidious onset in the fifth decade, with abnormal behavior and parkinsonian features. METHOD: We present the clinical features of 12 patients from a family with CJD associated with a point mutation at codon 183 of the prion protein gene. RESULTS: The mean age at onset was 44.0 Ý 3.7; the duration of the symptoms until death ranged from two to nine years. Behavioral disturbances were the predominant presenting symptoms. Nine patients were first seen by psychiatrists. Eight patients manifested parkinsonian signs. CONCLUSION: These clinical features bear a considerable resemblance to those described in FTDP-17

Post traumatic Parkinson's syndrom - a case report

A rare case of Parkinson's syndrome following head injury is described. The ocular abnormalties did not show any recovery. Early recognition of ocular changes helps to limit the imaging investigations to the cavernous sinus area. Aneurysm of the internal carotid artery, metastasis and trauma are the common aetiological factors in the few reported cases