Faciobrachial dystonic seizures are pathognomonic of leucine-rich glioma inactivated-1 (LGi1) antibody, non-paraneoplastic limbic encephalitis. Faciobrachial dystonic seizures usually precede limbic encephalitis by about a month. It is unknown whether, if untreated, faciobrachial dystonic seizures inevitably progress to limbic encephalitis. We present an LGi1 seropositive patient with a year’s ...

BACKGROUND Klinefelter's syndrome (KS) has been associated with tremor, but reports on tremor phenomenology and treatment are limited. CASE REPORTS Patient 1 is a 17-year-old male with a dystonic tremor treated with deep brain stimulation (DBS). Patient 2 is a 57-year-old male with a predominant left hand resting tremor and dystonic features. DISCUSSION Our cases suggest that the tremor in ...

Faciobrachial dystonic seizures (FBDS) are closely associated with antibodies (Ab) to the leucine-rich glioma inactivated-1 protein (LGI1) component of the voltage-gated potassium channel (VGKC) complex. They precede or indicate the onset of limbic encephalitis (LE). Immunotherapy can prevent the development of LE and cognitive impairment. FBDS are very brief, very frequent unilateral dystonic ...

Dystonic storm is a frightening hyperkinetic movement disorder emergency. Marked, rapid exacerbation of dystonia requires prompt intervention and admission to the intensive care unit. Clinical features of dystonic storm include fever, tachycardia, tachypnea, hypertension, sweating and autonomic instability, often progressing to bulbar dysfunction with dysarthria, dysphagia and respiratory failu...

turing of the left upper extremity. The dystonic posture of the left upper extremity caused levitation of the arm with flexed fingers and an extended wrist. Stimulussensitive generalized myoclonic jerks were Dear Sir, Creutzfeld-Jakob disease (CJD) is a progressive spongiform encephalopathy that is mainly characterized by rapidly progressive dementia, myoclonus, ataxia, epilepsia partialis cont...

Background Systematic perceptual distortions of tactile space have been documented in healthy adults. In isolated focal dystonia impaired spatial somatosensory processing is suggested to be a central pathophysiological finding, but the structure for different body parts has not previously explored. Objectives The objective this study was assess organization with novel behavioral paradigm distan...

INTRODUCTION A special group of focal dystonia is that known as occupational, which include dystonic disorders triggered by repetitive motor activity, closely associated with the professional activity of a specific task that the affected person performs. In this sense, musicians are a population particularly vulnerable to this disorder, which is presented during the execution of highly trained ...

A 5 year old girl with gross gastrooesophageal reflux had pronounced dystonic neck movements that resolved after fundoplication and resolution of the oesophagitis (Sandifer's syndrome). Oesophageal manometry before operation showed that peristalsis improved when she tilted her head to one side. Dystonic posturing may promote acid clearance from the distal oesophagus.

The paucity of literature in pediatric neurotransmitter diseases indicates a gap in our understanding of neurochemistry and the clinical phenomenology. However, the dramatic clinical responses to dopaminergic therapy in dopa-responsive dystonias opened avenues for rationalizing this therapeutic approach in other pediatric neurotransmitter disorders. This chapter aims to link up dopaminergic the...