The cause of objective tinnitus provoked by palatal and middle-ear myoclonus may be identified by magnetic resonance imaging of the central nervous system in the Guilliain-Mollaret triangle or it may be idiopathic. The idiopathic cases are rare. From the literature, one can perceive the following unanswered questions: (1) Are palatal and middle-ear myoclonus different entities or do they usuall...

BACKGROUND Myoclonus-dystonia is a condition that manifests predominantly as myoclonic jerks with focal dystonia. It is genetically heterogeneous with most mutations in the epsilon sarcoglycan gene (SGCE). In medically refractory cases, deep brain stimulation (DBS) has been shown to provide marked sustainable clinical improvement, especially in SGCE-positive patients. We present two patients wi...

A 22 year old patient with non-familial progressive myoclonus, macular cherry-red spot, moderate cerebellar syndrome and normal intelligence is described. The myoclonus began at the age of 18 years. Focal myoclonus could easily be elicited by voluntary and passive movements, and by touch and electrical stimulation of median nerve. Somatosensory evoked potentials showed a high voltage early comp...

BACKGROUND Paraneoplastic movement disorders in prostate cancer are rare, and to our knowledge paraneoplastic myoclonus has not previously been reported. CASE REPORT We report two men with adenocarcinoma of the prostate who developed isolated alcohol-responsive action myoclonus of one leg. Myoclonus was absent at rest but triggered by movement, standing, or walking. Evaluations excluded malig...

Pretreatment with 5-hydroxytryptophan (5-HTP), a precursor of 5-HT, antagonised while pretreatment with p-chlorophenylalanine (PCPA), a 5-HT depletor, potentiated the myoclonus induced by picrotoxin, a GABA antagonist. Pretreatment with aminooxyacetic acid (AOAA), a GABA transaminase inhibitor, antagonised picrotoxin-induced myoclonus. The combined effect of the least protective doses of AOAA a...

Despite having had normal speech until age 21 when he contracted varicella encephalitis, this 53-year-old man had always considered himself a stutterer. Neurologic examination revealed lightning-like facial and neck contractions during speech, which were absent at rest (video). Brain and cervical spine MRI had normal results. Surface EMG confirmed intermittent high-amplitude, short-duration bur...

BACKGROUND Acquired generalized repetitive myoclonus may be mistaken for tremor. Distinguishing myoclonus has etiologic and therapeutic implications. OBJECTIVE To describe isolated generalized polymyoclonus and the outcomes of etiologic evaluations at the time of diagnosis. DESIGN Computer search of the Mayo Movement Neurophysiology Laboratory database and medical records linkage system. ...

Background: Various movement disorders can be found in ataxia–telangiectasia (AT), including ataxia, dystonia, chorea, and myoclonus, but myoclonus has rarely been described as the predominant feature in AT. Case Report: We report two AT patients with prominent myoclonus, illustrating an unusual presentation of this disorder. Sequencing of the ATM gene in the first patient revealed a homozygous...