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This study analyzed the relationship between performance on the American Academy of Neurology Residency In-Service Training Examination (RITE) and subsequent performance on the American Board of Psychiatry and Neurology (ABPN) Certification Examination.

Hematopoietic stem cell transplantation (HSCT) may slow the progression of Krabbe disease (KD) if performed early in the disease. The authors' studies indicate that more than 90% of patients with KD have severe abnormalities in peripheral nerve conduction.

Krabbe disease (KD) is associated with marked central and peripheral demyelination and nerve conduction studies (NCS) typically show a mixed sensorimotor demyelinating peripheral neuropathy (PN).

We analyzed relationships among pubertal stage at disease onset, sex, disease severity, and acetylcholine receptor antibody (AChR Ab) levels in 46 patients with autoimmune juvenile myasthenia gravis (JMG). Female predominance was least in children with prepubertal disease onset (F:M 1.3:1) and increased in patients with peripubertal (F:M 1.8:1) and postpubertal (F:M 14:1) onset. Seronegative JMG was most common in children with early disease onset: 4 of 9 (44) with prepubertal, 4 of 22 (18) with peripubertal, and 0 of 15 (0) with postpubertal onset were seronegative. The rapid therapeutic response to plasmapheresis was useful in distinguishing some patients with seronegative JMG from those with congenital myasthenia gravis (CMG). The high frequency of seronegative JMG in patients with prepubertal onset indicates that AChR Ab assays do not adequately discriminate between JMG and CMG in young children. Furthermore, the different sex distribution in patients with different pubertal stages at disease onset suggests that sex hormones play an important modulating role in JMG.

We assessed the influence of race, sex, and puberty upon clinical features and outcome in 115 patients with autoimmune juvenile myasthenia gravis (JMG). These demographic variables influenced not only disease incidence but also disease severity, response to therapy, and outcome, despite comparable therapeutic strategies. Among white patients, those with prepubertal onset had low incidence and equal sex ratio; the incidence in females increased during and after puberty; males had lesser disease severity than females during and after puberty (p< 0.05); spontaneous remissions were most frequent (44, p= 0.001) and persistence of active JMG for more than 10 years was least frequent (p= 0.05) in patients with prepubertal onset; remissions were more frequent after early than late thymectomy (p= 0.03); and final disease severity was less after early than late thymectomy. Black patients had similar incidence, disease severity, and sex ratio (F:M = 2:1) with pre-, peri-, or postpubertal disease onset; infrequent spontaneous or treatment-induced remissions; and the same final disease severity after early or late thymectomy. These observations imply that race and sex hormones modify the clinical features and outcome of JMG; spontaneous remissions are common in white patients with prepubertal disease onset; early thymectomy may be more beneficial than late thymectomy in white patients; and the role of thymectomy in the youngest patients is uncertain. We suggest that demographic factors should be considered when evaluating past and future therapeutic strategies for JMG.

The authors reviewed the records of patients with myasthenia gravis who took cyclosporine for at least 6 months between November 1987 and January 1999. Of 57 patients who took cyclosporine for an average of 3.5 years, 55 (96) had clinical improvement. The median time to best clinical response was 7 months. Corticosteroids were discontinued or decreased in 95 of 38 patients taking them. Major side effects included elevated serum creatinine (28) and malignancy (11). Five percent could not afford or tolerate the drug.

The authors reviewed the incidence of cancer, repetitive nerve stimulation findings, and response to treatment in 73 patients with Lambert-Eaton myasthenic syndrome. Thirty-one patients (42) had lung cancer, 29 small cell. Doubling of the compound motor action potential amplitude in three tested distal muscles was seen in only 41 of patients. Treatment with 3,4-diaminopyridine produced moderate to marked self-reported functional improvement in 79 of the 53 treated patients.

Information is provided concerning the new subspecialty certificate in neuromuscular medicine of the American Board of Psychiatry and Neurology and the eligibility requirements for such certification of practicing neurologists and child neurologists. The Accreditation Council for Graduate Medical Education has approved fellowship training in the subspecialty, and it is likely that residents who wish to pursue a career in neuromuscular medicine will select this training option.

Serial single-fiber EMG (SF-EMG) studies in the frontalis muscle showed increased jitter with normal fiber density 15 days after facial nerve trauma. Both measurements were increased thereafter. The maximum increases occurred at 37 days, coincident with the initial return of function, and persisted until 67 days, when function had returned to normal. Thereafter, both features returned toward normal values, but were still elevated 34 months after injury.

The authors report the results of a prospective, placebo-controlled, randomized study to evaluate the effectiveness of 3,4-diaminopyridine (DAP) in patients with Lambert-Eaton myasthenic syndrome (LEMS) and to determine the acute and long-term side effects of DAP.