Spinal-generated movement disorders (SGMDs) include spinal segmental myoclonus, propriospinal myoclonus, orthostatic tremor, secondary paroxysmal dyskinesias, stiff person syndrome and its variants, movements in brain death, and painful legs-moving toes syndrome. In this paper, we review the relevant anatomy and physiology of SGMDs, characterize and demonstrate their clinical features, and pres...

Opsoclonus-myoclonus syndrome is a rare condition with dyskinesia of eye movements and myoclonic movements of the trunk and limbs. It is linked to malignancies, infections and other conditions. We present a case of post-vaccination opsoclonus-myoclonus syndrome. We believe that our patient also experienced a viral illness post-vaccination and prior to the development of opsoclonus-myoclonus. Th...

illness is benign, and the onset is usually in the 3rd decade of life. EMG revealed (20-50 msec) positive and (20-150 msec) negative myoclonus synchronized in antagonistic muscles. Major EEG abnormalities were generalized 4-6 Hz atypical spike and wave bursts and polyspikes. The P25-N33 amplitude of the median nerve SEP was increased (33.5 & 25.8 nV). EMG reflex myoclonic bursts from median ner...

Background Myoclonic jerks are due to sudden, brief, involuntary muscle contractions, positive myoclonus, or brief cessation of ongoing muscular activity, negative myoclonus, and may be difficult to recognize. Case Report We describe an immunocompetent, adult, male patient with sleep-related, multifocal, myoclonic jerks and neurotoxoplasmosis with abnormal cerebrospinal fluid but normal brain...

A patient with progressive muscular rigidity associated with reflex myoclonus is described. The muscular rigidity was predominantly axial, and the myoclonic jerks affected axial and leg muscles. Jerks occurred either spontaneously, or in response to touch to the perioral region, or to stretch of head and neck muscles. Physiological investigations suggested that the myoclonus originated in the m...

DYT11 myoclonus-dystonia (M-D) is a movement disorder characterized by myoclonic jerks with dystonic symptoms and caused by mutations in paternally expressed SGCE, which codes for ε-sarcoglycan. Paternally inherited Sgce heterozygous knock-out (KO) mice exhibit motor deficits and spontaneous myoclonus. Abnormal nuclear envelopes have been reported in cellular and mouse models of early-onset DYT...

Three patients are described with nonrhythmic repetitive axial myoclonic jerks causing symmetric flexion of the neck, trunk, hips and knees. No electrophysiological evidence of a cortical or brainstem reticular origin for the myoclonus was found. In the first patient the axial jerks only occurred spontaneously. The latencies of recruitment of spinal segments during a jerk indicated that the dis...

The causal disease Progressive myoclonus epilepsy of the Unverricht-Lundborg type (EPM1) is an autosomal recessive neurodegenerative disorder that has the highest incidence among the progressive myoclonus epilepsies worldwide (Berkovic et al. 1986; Marseille Consensus Group 1990). It is characterized by stimulussensitive myoclonus, and tonic-clonic epileptic seizures. As EPM1 progresses, patien...

background: alveolar hypoxia induces monophasic pulmonary vasoconstriction in vivo, biphasic vasoconstriction in the isolated pulmonary artery, and controversial responses in the isolated perfused lung. pulmonary vascular responses to sustained alveolar hypoxia have not been addressed in the isolated perfused rat lung. in this study, we investigated the effect of sustained hypoxic ventilation o...

West Nile virus (WNV) is an arthropod borne neurotropic single stranded RNA flavivirus with <1% developing presenting with neurological disease. Immunocompromised and elderly patients are more prone to developing WNV meningitis or encephalitis. Definitive diagnosis of WNV meningoencephalitis is a combination of clinical suspicion and cerebrospinal fluid (CSF) serology. Forty-eight year old Cauc...