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Myoclonus in corticobasal degeneration (CBD) has often been associated with severe and difficult to treat disabilities. Levetiracetam is a new antiepileptic agent with antimyoclonic effects. Herein, we present a 72-year-old woman with clinically probable CBD and with spontaneous rhythmic myoclonus
Levetiracetam (LEV) has been shown to suppress myoclonus of various origins. Corticobasal degeneration (CBD), a progressive neurodegenerative disorder with Parkinsonian syndrome, is frequently accompanied by myoclonus. We investigated the effect of LEV on myoclonus in two CBD patients. LEV
We report a new family with palatal myoclonus, pyramidal tract signs, cerebellar signs, marked atrophy of the medulla oblongata and spinal cord, and autosomal dominant inheritance. These findings were almost identical with those in patients previously reported to have histopathologically confirmed
Four autopsied cases of myoclonus, ataxia, and epilepsy from 2 separate pedigrees are described. An identical pattern of focal brainstem lesions was found in all the cases with selective and symmetrical degeneration of the dentate and second order somatosensory nuclei. The combined clinical and
A 23-year-old man presented with a history characterized by a myoclonic syndrome developing over a period of seven years. Predominant symptoms were intention and activity myoclonus, generalized epileptic seizures occurring infrequently from the age of 20, a slowly progressive cerebellar syndrome
We studied four patients with familial olivopontocerebellar atrophy (OPCA) who had abnormal twitching of the cheeks and perioral muscles induced by facial movements. With the muscles at rest, electromyographic (EMG) recordings of the orbicularis oris and risorius muscles revealed myokymic discharges
We prospectively studied the clinical and electrophysiological features of myoclonic tremulous movements in patients with multiple system atrophy (MSA). Among 42 consecutive patients, 12 MSA-p (parkinsonian type) and 3 MSA-c (cerebellar type) patients showed small-amplitude myoclonic movements.
The neuropathologic findings in an idiopathic case of the opsoclonus/myoclonus syndrome are reported. Although neurologic dysfunction may have been more widespread, structural lesions were limited to the cerebellum and inferior olives. Severe depletion of Purkinje cells with preservation of granular
We report a 13-year-old girl with dentatorubal-pallidoluysian atrophy (DRPLA), presenting clinically as progressive myoclonic epilepsy. The action myoclonus, which severely impaired her daily life, was markedly improved by administration of piracetam, a drug reportedly useful for myoclonus of
A 19-year-old man developed progressive unsteady gait and speech disturbance two years ago. He recently noticed muscle twitches in the left limb. Neurological examination revealed scanning speech, saccadic ocular movement, ataxia of trunk and limbs predominant on the left side. Rhythmic myoclonus
A 22-year-old female with progressive myoclonus epilepsy (PME) considered to be due to hereditary dentato-rubro-pallido-luysian atrophy (DRPLA) was reported. Some of her family members showed progressive myoclonus, seizures, dementia, ataxia and choreoathetosis, with variation of onset from
Five patients with unilateral myoclonus and a clinical diagnosis of corticobasal degeneration (CBD) were studied. All patients showed enhanced long-loop responses in their myoclonic arms without enlarged somatosensory potentials. The cortical relay time of the long-loop responses was studied in
A single case study of a 58 year-old male with right asymmetric apraxia and akinetic-rigid syndrome is described. Brainimaging scans (MRI, SPECT) indicated asymmetric cortical atrophy compatible with the diagnosis of Corticobasal Degeneration. Reflex myoclonus was absent and myoclonic discharges
Focal reflex myoclonus was observed in a patient clinically diagnosed as affected by corticobasal degeneration. Myoclonus was not preceded by a cortical discharge and contralateral parietal components of the somatosensory evoked potentials were reduced in amplitude. No simple pathophysiological
A 66-year-old man with clinically diagnosed corticobasal degeneration was studied electrophysiologically. The patient had bilateral forced grasping, rigidity, bradykinesia and hyperreflexia which were predominant on the right side, motor aphasia, constructional apraxia, forced laughing,