Progressive encephalomyelitis with rigidity and myoclonus (PEWR) is a rare neurological disorder, characterised by muscular rigidity, painful spasms, myoclonus, and evidence of brain stem and spinal cord involvement. A 73-year-old white man was admitted with a 10-day history of painful muscle spasms and continuous muscle rigidity on his left lower limb. He had involuntary spasms on his legs and...

113 cases of infantile spasms were diagnosed in the period 1 April 1970 to 31 March 1975 after Denmark changed her immunization programme. Previously whooping cough vaccine was given as the triple combination at 5, 6, and 15 months of age. From 1 April 1970 it was given as a monovalent vaccine at 5 and 9 weeks of age and at 10 months of age. A comparison with previous material of 86 cases of in...

This prospective, open label, uncontrolled study was performed to evaluate the efficacy and tolerability of the modified Atkins diet in children with refractory infantile spasms. Fifteen consecutive children aged six months to three years having daily infantile spasms in clusters with electroencephalographic evidence of hypsarrhythmia despite treatment with hormonal treatment (oral corticostero...

BACKGROUND Voluntary nystagmus has been recognized as a pendular, rapid, conjugate, primarily horizontal, benign eye movement initiated and maintained by voluntary effort. CASE A 10-year-old Japanese girl presented with voluntary nystagmus associated with accommodation spasms. Her chief complaints, intermittent blurred vision, headache, and soreness of the eyes, were thought to be related to ...

Although coronary artery spasm has been implicated as an important cause of myocardial ischemia in humans, an animal model of reversible segmental coronary constriction has not been described. To provoke coronary spasm in open-chest dogs, selected vasoconstricting agents adsorbed to viscous ion exchange gels were applied topically to the surface of epicardial coronary arteries. The procedure pr...

PURPOSE Epileptic spasms sometimes begin after the first year of life, and such seizures are recognized as late-onset spasms (LOS). The prognosis of LOS is poor, and a treatment strategy has not been established. This study aimed to assess the short- and long-term effects of adrenocorticotropic hormone (ACTH) therapy for LOS. METHODS We investigated the rate of LOS in 22 patients (14 boys and...

BACKGROUND The use of adrenocorticotrophic hormone (ACTH) and prednisolone in the management of infantile spasms has been well established, but is associated with significant morbidity and cannot be used as long-term medication. Since the introduction of vigabatrin, results have been promising with suggestions currently that it should be used as first-line management of infantile spasms. AIM ...

OBJECTIVE The clinical and electrographic features of seizures in anti-LGI1 encephalitis are distinct from those seen in other autoimmune encephalitides or non-encephalitic epilepsies. One electroclinical phenomenon specific to the condition consists of lateralized motor spasms, known as faciobrachial dystonic seizures (FBDS). An electrodecremental pattern overriding a "DC shift" has been descr...