objective: hashimoto encephalopathy (he) is known as a steroid-responsive encephalopathy associated with autoimmune thyroiditis or nonvascular inflammation-related autoimmune meningoencephalitis. the average age of onset of he is approximately 50 years; and it is more common in women. the onset of he may be acute or subacute. the course of most he cases is relapsing and remitting, which is simi...

The phenotypic spectrum of STXBP1-related encephalopathy ranges from infantile epileptic to intellectual disability with nonsyndromic or absent epilepsy. Although being frequently reported, the tremor associated STXBP1 has not been fully characterized date. aim our study was describe it. We recruited patients due variants, regardless their phenotype, who had at examination and underwent neuroph...

A 20-year-old woman with subacute sclerosing panencephalitis, bedridden because of dystonic posturing and very frequent myoclonic involuntary movements, improved dramatically with carbamazepine. The favorable effect of the carbamazepine on myoclonus was demonstrated by discontinuing and reintroducing carbamazepine, and videoing the consequent reappearance and disappearance of the myoclonus. [Pu...

In a case of acute disseminated encephalomyelitis displaying opsoclonus-myoclonus syndrome, magnetic resonance images (MRI) disclosed pontine lesions involving part of the pontine paramedian reticular formation and the raphe nucleus. Shrinkage of the lesions correlated with the patient's clinical state. This observation supports the hypothesis that a pontine lesion can cause opsoclonus-myoclonu...

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...