PURPOSE Numerous studies have suggested that the ketogenic diet is effective in the treatment of epileptic spasms, even in refractory cases. However, there has been very limited demonstration of prompt and complete (video-EEG confirmed) response. We set out to describe our center's experience with the ketogenic diet in the treatment of children with highly refractory epileptic spasms, with rigo...

PURPOSE ACTH is currently the standard first-line therapy for new-onset infantile spasms, but it has significant side effects. We hypothesized the ketogenic diet (KD), previously reported as beneficial for intractable infantile spasms, would have similar efficacy, but better tolerability than ACTH when used first-line. METHODS We conducted a retrospective chart review of all infants started o...

The brain stem provides most of the noradrenaline (NA) present in the spinal cord, which functions to both increase spinal motoneuron excitability and inhibit sensory afferent transmission to motoneurons (excitatory postsynaptic potentials; EPSPs). NA increases motoneuron excitability by facilitating calcium-mediated persistent inward currents (Ca PICs) that are crucial for sustained motoneuron...

UNLABELLED Dystonias are organic central motor processing disorders characterized by involuntary muscular contractions or incontrollable spasms induced by task-specific movements. Adduction laryngeal dystonias present with important speech impairments, with inappropriate spasms and abrupt voice breaks. The diagnosis is based on clinical features, evaluation by a speech therapist and transnasal ...

PURPOSE We retrospectively investigated the feasibility and clinical efficacy of balloon dilatation and subsequent retrievable stent insertion, when necessitated, for pyloric spasms after pylorus-preserving gastrectomy (PPG). MATERIALS AND METHODS Forty-five patients experiencing pyloric spasms after PPG underwent fluoroscopic balloon dilations to alleviate obstructive symptoms due to delayed...

Spasmodic dysphonia (SD) is a voice disorder characterized by abnormal intermittent spasms of intralaryngeal muscles that result in voice breaks during speech. In the adductor variant of spasmodic dysphonia (ADSD), spasms of the adductor muscles cause strangled voice breaks and a strained-strangled voice quality. In the abductor variant (ABSD), spasms of the posterior cricoarytenoid muscle (PCA...

Four patients had a chronic progressive disorder beginning in middle age and involving stiffness and painful spasms of the lower limbs. Spasms were spontaneous, reflex, and induced by voluntary movement. Patients had rigidity and abnormal postures of one or both legs. There was no truncal rigidity or exaggerated lumbar lordosis. Despite the presence of symptoms for up to 16 years, symptoms and ...

Infantile spasms are a catastrophic form of pediatric epilepsy with inadequate treatment. In patients, mutation of ARX, a transcription factor selectively expressed in neuronal precursors and adult inhibitory interneurons, impairs cell migration and causes a major inherited subtype of the disease X-linked infantile spasms syndrome. Using an animal model, the Arx((GCG)10+7) mouse, we determined ...

BACKGROUND AND PURPOSE Although repetitive involuntary movements are a well-recognized manifestation of carotid occlusive disease, similar movements have not been reported with a lacunar infarction outside of the basal ganglia or subthalamic nucleus. We describe a man with tonic spasms associated with a lacunar infarction in the right ventral pons. CASE DESCRIPTION Involuntary tonic spasms of...

jaws locked, though some power of deglutition was still retained; the spasms were frequent and pretty violent; the trunk, however, did not very markedly bend itself; the muscles of the extremities were greatly affected, and the abdominal recti were stiff and in constant spasmodic contraction." The Baboo prescribed tincture of Cannabis Indica and spirits of chloroform ; also chloral hydrate and ...