Anti-CV2/CRMP5 Paraneoplastic Chorea Effectively Managed with Intravenous Amantadine.

Background: Paraneoplastic chorea is typically a subacute progressive hyperkinetic movement disorder. The mainstay of treatment is managing the underlying neoplasm. However, the clinical course may be variable, and effective symptomatic management can precede the start of cancer treatment. Case report: A 63-year-old man presented with insidious onset, slowly progressive generalized chorea for 1 year, later diagnosed as anti-CV2/CRMP5 autoantibody positive paraneoplastic chorea. His chorea was markedly improved with intravenous amantadine. Discussion: In patients with anti-CV2/CRMP5 autoantibody-related chorea, sequential follow-up of brain magnetic resonance imaging reveals progression from active inflammation to atrophy. Our report highlights the efficacy of intravenous amantadine in paraneoplastic chorea.

Comparison of gait parameters between drug-naïve patients diagnosed with multiple system atrophy with predominant parkinsonism and Parkinson's disease.

INTRODUCTION: Even though gait symptoms are prominent in patients diagnosed with multiple system atrophy with predominant parkinsonism (MSA-P) compared with Parkinson's disease (PD), the gait patterns of MSA-P were not clearly elucidated. We investigated postural instability and gait disturbances in MSA-P compared with PD. METHODS: We enrolled 34 drug-naïve patients with PD and 26 with MSA-P, and 18 normal controls in this study. Parkinsonism was evaluated by the Unified Parkinson's disease rating scale (UPDRS) part 3 and cognition was assessed with mini-mental status exam (MMSE). All the enrolled subjects underwent Pedoscan and GAITRite to objectively measure postural stability and gait. We compared the results of posturography and gait analysis among 3 groups, and performed correlation analysis of gait parameters with MMSE, UPDRS part 3 and posturography results. RESULTS: No difference was detected in demographic and clinical variables, except tremor sub-score of UPDRS part 3, urinary symptoms and orthostatic hypotension. MSA-P patients showed larger total anterior-posterior and lateral movement of centre of pressure (COP), and widened base of support than PD patients. In correlation analysis, MMSE score, axial sub-score of UPDRS part 3 and lateral movement of COP were correlated with gait parameters in PD patients, while only axial sub-score was associated in MSA-P patients after controlling for age, sex, height, body weight, education year, and disease duration. CONCLUSION: Even at an early stage, MSA-P patients demonstrated more postural instability and gait disturbance compared with PD patients, and the related factors with gait disturbance in PD and MSA-P might be different.

Severe Hemifacial Spasm is a Predictor of Severe Indentation and Facial Palsy after Microdecompression Surgery.

BACKGROUND AND PURPOSE: Hemifacial spasm (HFS) is mostly caused by the compression of the facial nerve by cerebral vessels, but the significance of spasm severity remains unclear. We investigated the clinical significance of spasm severity in patients with HFS who underwent microvascular decompression (MVD). METHODS: We enrolled 636 patients with HFS who underwent MVD between May 2010 and December 2013 at Samsung Medical Center (SMC), Seoul, Korea. Subjects were divided into two groups based on spasm severity: severe (SMC grade 3 or 4) and mild (SMC grade 1 or 2). We compared demographic, clinical, and surgical data between these two groups. RESULTS: The severe-spasm group was older and had a longer disease duration at the time of MVD compared to the mild-spasm group. Additionally, hypertension and diabetes mellitus were more common in the severe-spasm group than in the mild-spasm group. Regarding surgical findings, there were more patients with multiple offending vessels and more-severe indentations in the severe-spasm group than in the mild-spasm group. Even though the surgical outcomes did not differ, the incidence of delayed facial palsy after MVD was higher in the severe-spasm group than in the mild-spasm group. Logistic regression analysis showed that severe-spasm was correlated with longer disease duration, hypertension, severe indentation, multiple offending vessels, and delayed facial palsy after MVD. CONCLUSIONS: Spasm severity does not predict surgical outcomes, but it can be used as a marker of pathologic compression in MVD for HFS, and be considered as a predictor of delayed facial palsy after MVD.