ABSTRACT
Orthostatic hypotension is a frequent symptom in patients with multiple system atrophy and it has an important impact on their quality of life. We report a case of idiopathic orthostatic hypotension, in a patient with multiple system atrophy (Shy-Drager syndrome), treated with pacemaker implantation resulting in a substantial improvement in the quality of his life.
Hipotensão ortostática é um sintoma freqüente em pacientes com atrofia de múltiplos sistemas e tem importante impacto na sua qualidade de vida. Relatamos um paciente com hipotensão ortostática idiopática e atrofia de múltiplos sistemas (Síndrome de Shy-Drager), tratado com implantação de marca-passo resultando numa evidente melhora na qualidade de vida.
Subject(s)
Humans , Male , Middle Aged , Multiple System Atrophy/diagnosis , Hypotension, Orthostatic/diagnosis , Hypotension, Orthostatic/therapy , Pacemaker, Artificial , Shy-Drager Syndrome , Neurodegenerative DiseasesABSTRACT
This study evaluates the diagnostic value of morphometric magnetic resonance imaging (MRI) in the differential diagnosis among Parkinson's disease (PD), progressive supranuclear palsy (PSP) and multiple system atrophy (MSA). We studied 21 PD cases, 11 MSA-c, 8 MSA-p and 20 PSP cases. Midbrain area (Ams), pons area (Apn), middle cerebellar peduncle (MCP) and superior cerebellar peduncle (SCP) were measured using MRI. Comparisons were made between PD, MSA-p, MSA-c and PSP. Apn, MCP and SCP morphometry dimensions presented differences among groups. Ams below 105 mm² and SCP smaller than 3 mmwere the most predictive measures of PSP (sensitivity 95.0 and 80.0 percent, respectively). For the group of MSA-c patients, Apn area below 315 mm² showed good specificity and positive predictive value (93.8 percent and 72.7 percent, respectively). In conclusion, dimensions and cut off values obtained from routine MRI can differentiate between PD, PSP and MSA-c with good sensitivity, specificity and accuracy.
Morfometria pela ressonância magnética (RM) no diagnóstico diferencial entre doença de Parkinson (DP), paralisia supranuclear progressiva (PSP) e atrofia de múltiplos sistemas (AMS). Este estudo avaliou a RM no diagnóstico diferencial de 21 casos com DP, 11 AMS-c, 8 AMS-p e 20 com PSP. A área sagital do mesencéfalo (Ams), área sagital da ponte (Apn), largura do pedúnculo cerebelar médio (PCM) e pedúnculo cerebelar superior (PCS) foram medidas pela RM e realizadas comparações entre destes pacientes. A Ams <105 mm² e a largura média do PCS <3 mm foram preditivas para PSP (sensibilidade de 95,0 e 80,0 por cento, respectivamente). Nos casos de AMS-c a área pontina <315 mm² apresentou boa especificidade e valor preditivo positivo para o diagnóstico (93,8 por cento e 72,7 por cento). Em conclusão, as dimensões e valores de cortes obtidos a partir da RM podem diferenciar PD, PSP e AMS-c, com sensibilidade, especificidade e precisão.
Subject(s)
Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Magnetic Resonance Imaging/methods , Mesencephalon/pathology , Multiple System Atrophy/diagnosis , Parkinson Disease/diagnosis , Supranuclear Palsy, Progressive/diagnosis , Cross-Sectional Studies , Diagnosis, Differential , Multiple System Atrophy/pathology , Predictive Value of Tests , Parkinson Disease/pathology , Sensitivity and Specificity , Supranuclear Palsy, Progressive/pathologyABSTRACT
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.
Subject(s)
Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Cerebellum/pathology , Magnetic Resonance Spectroscopy/methods , Multiple System Atrophy/diagnosis , Parkinsonian Disorders/diagnosis , Supranuclear Palsy, Progressive/diagnosis , Atrophy , Case-Control Studies , Diagnosis, Differential , Double-Blind Method , Prospective Studies , Statistics, NonparametricABSTRACT
Objective: to detect the value of mid-sagittal MRI measurements of the midbrain and pons in the diagnosis of Progressive Supranuclear Palsy [PSP] and to differentiate it from mimics [Parkinson Disease [PD] and Multiple-System Atrophy of the Parkinson Type [MSA-P]], and to what extend these radiological measurements correlate with the clinical aspects of PSP
Methods: MRI of 13 patients with PSP, 25 patients with PD, and 10 patients with MSA-P, as well as 20 age-matched controls were prospectively studied. The areas of the midbrain tegmentum and the pons were measured on mid-sagittal MRI using the display tools. The ratio of the area of the midbrain to the area of the pons was also evaluated in all subjects. Patients were also evaluated clinically using Hoehn and Yahr Scale, UPDRS and MMSE scale in addition to full general, neurological assessment and routine laboratory investigations
Results: the average midbrain area of the patients with PSP [57 mm[2]] was significantly smaller than that of the patients with PD [101 mm[2]] and MSA-P [97.9 mm[2]] and that of the age-matched control group [116.9 mm[2]]. There was no overlap between patients with PSP and patients with PD or normal control subjects regarding midbrain area values. However, patients with MSA-P showed some overlap of the values of individual areas with values of individual areas with values from patients with PSP. The ratio of the area of the midbrain to the area of pons in patients with PSP [0.123] was significantly smaller than that in those with PD [0.207] and MSA-P [0.265] and in normal control subjects [0.235]. Also, use of the ratios allowed differentiation between PSP groups and the MSA-P group. Good significant correlation was observed between various radiological measurements studied and disease duration, staging and severity
Conclusion: the mid-sagittal MRI measurements of the midbrain area can differentiate between the PSP from PD, MSA-P and normal aging and these radiological measurements correlated well with the clinical aspects of the syndrome of PSP
Subject(s)
Humans , Male , Female , Magnetic Resonance Imaging , Parkinson Disease/diagnosis , Multiple System Atrophy/diagnosisABSTRACT
A síndrome de Shy-Drager é uma doença rara descrita em 1960, que consiste em falência progressiva do sistema nervoso autônomo, com parkinsonismo e hipotensão ortostática. Os autores fazem uma revisão, relatando um caso e mostrando aspectos atuais sobre diagnóstico e tratamento.