Background Hereditary myoclonus dystonia is often due to changes in the SGCE gene. Dystonia (DYT)-SGCE has a variable phenotype that can involve focal or generalized myoclonus and various forms of task-specific, segmental, or generalized dystonia. Psychiatric comorbidities are common. Case Report We report a case of a young woman with generalized myoclonus, dystonia, and intellectual disabili...

Spinal segmental myoclonus is defined as a rare involuntary movement characterized by myoclonic jerks of spinal origin. We describe the case of a 62-year-old woman who developed spinal segmental myoclonus 4 months after undergoing cervical laminoplasty for ossification of the posterior longitudinal ligament. Myoclonic jerks were observed in the upper trapezius innervated by C3-4, which correspo...

Two cases of spinal myoclonus are described; in both patients myoclonus was responsive to stimuli and absent during sleep. The first patient was considered to have viral neuronitis and the condition resolved spontaneously. The second patient had spinal cord ischaemia; there was electro-physiological evidence of abnormal alpha motor neurone activity and histological study of the spinal cord reve...

A 61-year-old woman developed acute dyslalia, dysphonia, dysphagia, and facial rhythmic jerks 8 hours after the intake of 2 tablets of metoclopramide 10 mg, prescribed for nausea during respiratory infection. Examination revealed dysphonia, dyslalia, dysphagia, and myoclonus in the orbicularis oculi (video 1 on the Neurology® Web site at Neurology.org), orbicularis oris, and palatopharyngeal (v...

Background: In this case report, we describe an unusual case of a patient with myoclonus only occurring during menses. Case Report: A 41-year-old female, known to have neurological sequelae after a car accident 1 year earlier, presented with myoclonic movements of the right arm and hand only during menses. Brain magnetic resonance imaging is compatible with head trauma. Electromyography shows b...

Among paraneoplastic neurologic disorders (PND), opsoclonus-myoclonus syndrome, so-called "dancing eye syndrome," is a rare disorder combining multivectorial eye movements, involuntary multifocal myoclonus, and cerebellar ataxia. Although several paraneoplastic antibodies against postsynaptic or cell-surface antigens have been reported, usually most patients are serum antibody negative. We repo...

Progressive supranuclear palsy (PSP), a neurodegenerative disease with the parkinsonism, is characterized by supranuclear eye movement disorders and static reflex disorder appearing in the neck / trunk from early period of muscle rigidity and illness. Myoclonus is the lightning involuntary muscle shrinkage resulting from excessive neurologic excitement. This disease develops with various condit...

The origin of myoclonus in patients with complex regional pain syndrome (CRPS) is unknown. Eight patients with CRPS related myoclonus were clinically evaluated and studied with intermuscular and corticomuscular coherence analysis. Jerks were present at rest, aggravated during action and were frequently associated with tremulousness or dystonia. Electromyography demonstrated a burst duration ran...

Four patients with cortical myoclonus were studied. All had reflex muscle jerking and grossly enlarged somatosensory evoked responses (SEPs) following electrical stimulation of the digital nerves. In addition, three of the patients had spontaneous or action-induced myoclonus. Back-averaging the EEG from these spontaneous muscle jerks showed a large positive wave over the contralateral somatomot...

BACKGROUND Niemann-Pick type C (NP-C) is a rare autosomal recessive progressive neurodegenerative disorder caused by mutations in the NP-C 1 or 2 gene. Besides visceral symptoms, presentation in adolescent and adult onset variants is often with neurological symptoms. The most frequently reported presenting symptoms of NP-C in adulthood are psychiatric symptoms (38 %), cognitive decline (23 %) a...