Multiple System Atrophy Of Cerebellar Type Research Articles

Arginine test is not reliable for diagnosing cerebellar multiple system atrophy

We evaluated the arginine growth hormone (GH) stimulation test for the diagnosis of cerebellar-type multiple system atrophy (MSAc) in patients with ataxia. Fourteen subjects with MSAc, 11 with idiopathic late-onset cerebellar ataxia (ILOCA), 10 with familial ataxia, and 10 healthy controls were tested. After pituitary GH deficiency was excluded, subjects underwent arginine testing. Peak serum GH response was analyzed. No significant differences in peak GH response were found between subject populations. Thirty-three percent of MSAc subjects mounted responses >10 microg/l GH. Thirty-six percent of ILOCA subjects and 40 percent of healthy controls mounted responses <4 microg/l GH. Arginine thus appears to be unreliable for the diagnosis of MSAc.

Frequency and Correlates of Involuntary Emotional Expression Disorder in Parkinson's Disease

Frequency and Correlates of Involuntary Emotional Expression Disorder in Parkinson's Disease

MR-morphometry in multiple system atrophy and sporadic adult onset ataxias of unknown aetiology – different approaches

Multiple system atrophy (MSA) is a sporadic, adult-onset disease characterized by neurodegeneration in basal ganglia, brainstem, cerebellum and intermediolateral cell columns of the spinal cord. Clinically, a parkinsonian (MSA-P) and a cerebellar type of MSA (MSA-C) are distinguished. Sporadic adult onset ataxias of unknown aetiology (SAOA) denote all non-hereditary degenerative adult onset ataxias distinct from MSA-C, but early differential diagnosis is often difficult. Using voxel-based morphometry (VBM) we found previously cerebellar grey and white matter loss (GML/WML) in MSA and SAOA, whereas brainstem (WML), basal ganglia and several cortical regions (GML) were only affected in MSA. To further investigate white matter pathology (WMP) in MSA and SAOA we used diffusion-tensor-imaging (DTI) and correlated changes with clinical data.

Early Development of Autonomic Dysfunction May Predict Poor Prognosis in Patients With Multiple System Atrophy

Multiple system atrophy (MSA) is diverse in clinical phenotype, disease progression, and prognosis. Sudden death is a leading cause of death in patients with MSA. To determine what clinical factors affect the progression and survival prognosis of those with MSA. A retrospective review of the medical records of 49 consecutive Japanese patients with pathologically confirmed MSA (29 men and 20 women; mean +/- SD age at onset, 59.8 +/- 6.5 years). Cox proportional hazards models were used to compare the risks of being in a wheelchair-bound state, being in a bedridden state, and having a shorter survival. Thirty-one patients were diagnosed as having cerebellar type MSA, and 18 were diagnosed as having parkinsonian type MSA. Twenty-nine patients with cerebellar type MSA and 17 patients with parkinsonian type MSA had autonomic dysfunction. The median times from disease onset to being in a wheelchair-bound state, being in a bedridden state, death, and the development of autonomic dysfunction were 3.5, 5.0, 7.0, and 2.5 years, respectively. Patients with an early development of autonomic dysfunction (within 2.5 years from the onset of MSA) had significantly higher risks of being in a wheelchair-bound state (multivariate-adjusted hazard ratio [HR], 4.32; 95% confidence interval [CI], 2.04-9.15), being in a bedridden state (HR, 3.87; 95% CI, 1.77-8.48), having a shorter survival (HR, 3.40; 95% CI, 1.61-7.15), and sudden death (HR, 7.22; 95% CI, 1.49-35.07). The early development of autonomic dysfunction is an independent predictive factor for rapid disease progression and shorter survival in patients with MSA.

Voxel-based analysis of multiple-system atrophy of cerebellar type: complementary results by combining voxel-based morphometry and voxel-based relaxometry

Voxel-based relaxometry (VBR) is a novel morphometric method that analyses the relaxation rate R2 derived from multi-echo T2-weighted images on a voxel-by-voxel basis. We used VBR to study the brain morphology of 14 patients suffering from multiple-system atrophy of cerebellar type (MSA-C) and compared the results with those obtained by voxel-based morphometry (VBM) of T1-weighted images. VBR analysis revealed reduction of relaxation rate R2 in the cerebellum and brainstem reflecting infratentorial brain atrophy. The affected regions largely corresponded to those regions in which VBM showed reductions of grey and white matter. In addition, R2 was increased in the putamen, a region in which VBM did not show abnormalities. Our data show that the combination of VBR and VBM provided convergent and complimentary information about the brain morphology of MSA-C.

Blepharospasm associated with multiple system atrophy: a case report and review of the literature

Although blepharospasm has been occasionally associated with parkinsonism, it has rarely been reported in patients with multiple system atrophy (MSA). We report a 65-year-old woman with MSA who developed blepharospasm seven years after onset, rendering her functionally blind. Clinical course and the findings of magnetic resonance imaging indicated cerebellar type MSA. The blink reflex studies showed prolonged R2 response and enhanced recovery cycle, indicating an increased excitability of the brainstem interneurons. These results suggest that pathophysiology of blepharospasm in MSA is similar to that of essential blepharospasm. Recognition of blepharospasm in MSA patients is important, as blepharospasm is a treatable feature in this otherwise intractable disorder.

Evoked potentials in multiple system atrophy (MSA).

To study the involvement of pyramidal tracts and sensory pathways in multiple system atrophy (MSA). Evoked potential studies were performed in 45 MSA patients suffering from either MSA of cerebellar type (MSA-C) or MSA of parkinsonian type (MSA-P). Motor evoked potentials were normal in all MSA patients, whereas visual and somatosensory evoked potential abnormalities were found in about 40% of the MSA patients with no significant difference between the cerebellar (MSA-C) and parkinsonian (MSA-P) subgroup. Abnormal latencies of wave III in brainstem auditory evoked potentials were significantly more frequent in MSA-C. Abnormalities of somatosensory, visual and auditory evoked potentials are frequent findings in MSA, whereas abnormal motor evoked potentials are not a characteristic feature of the disease.