RESUMEN
OBJECTIVE: Holmes tremor (HT) comprises rest, postural and intention tremor subtypes, usually involving both proximal and distal musculature. Perturbations of nigro-striatal pathways might be fundamental in the pathogenesis of HT along with cerebello-thalamic connections. METHODS: Nine patients with an HT phenotype secondary to thalamic stroke were included. Epidemiological and clinical records were obtained. Structural and functional brain imaging were performed with magnetic resonance imaging (MRI) or computed tomography (CT) and positron emission tomography (PET), respectively. Levodopa was administered in sequentially increasing dosage, with various other drugs in case of inadequate response. Longitudinal follow-up was performed for at least three months. The essential tremor rating assessment scale (TETRAS) was used for assessment. RESULTS: The mean latency from stroke to tremor onset was 50.4 ± 30.60 days (range 21-90 days). Dystonia was the most frequently associated hyperkinetic movement (88.8%). Tremor was bilateral in 22.2% of participants. Clinical response was judged based on a reduction in the TETRAS score by a prefixed value (≥ 30%), pertaining to which 55.5% (n = 5) of subjects were classified as responders and the rest as non-responders. The responders showed improvement with significantly lower doses of levodopa than the remaining nonresponders (240 ± 54.7 mg vs. 400 ± 40.8 mg; p = 0.012). CONCLUSION: Although levodopa is useful in HT, augmenting the dosage of levodopa beyond a certain point might not benefit patients clinically. Topography of vascular lesions within the thalamus might additionally influence the phenomenology of HT.
RESUMEN
Moyamoya disease (MMD) is a progressive steno-occlusive vasculopathy at the circle of Willis, characteristically involving the supra-clinoid segment of internal carotid arteries and their proximal branches with prominent collateral artery formation. Here we present, a series of imaging findings and varied clinical presentation of eight cases (n = 8) of MMD in the pediatric population (aged 0-18 years) after a retrospective review and detailed clinical evaluation. Detailed clinical history and examination were performed of eight pediatric patients of MMD with varied presentations. Magnetic resonance imaging (MRI) of the brain along with time of flight (TOF) sequence magnetic resonance angiography (MRA) was performed for all patients with a 3 T ((T) SEIMENS MRI scanner. Three out of eight cases (37.5%) presented with imaging findings of acute parenchymal infarction involving one or more major vascular territories showing diffusion restriction on DWI (diffusion-weighted image) sequence. Two of the patients (25%) showed chronic infarction with areas of gliosis and encephalomalacia. One child presented with watershed areas of infarction involving bilateral parieto-occipital region. In one of the patients (12.5%) being evaluated for dystonia, the only parenchymal finding detected was that of asymmetric ventriculomegaly. A solitary patient being evaluated for intermittent focal seizure followed by Todd's hemiparesis had normal parenchymal brain imaging. Leptomeningeal pattern of enhancement was noted in one patient (12.5%). Although predominantly an intracranial vasculopathy, MMD may have myriad presentations apart from stroke, some of which are highlighted in this series.
