Bruce O. Berg

Giant axonal neuropathy

SummaryGiant axonal neuropathy (GAN) is a disease characterized by a slowly progressive neuropathy and signs of central involvement, manifested by visual impairment, corticospinal tract dysfunction, ataxia, and dementia. Pathological hallmarks of the disease include axonal swellings packed with neurofilaments in both peripheral and central nervous systems, and accumulations of intermediate filaments in Schwann cells, fibroblasts, melanocytes, endothelial, and Langerhans cells. Rosenthal fibers, sometimes appearing in masses and mimicking Alexanders disease, emerge as a conspicuous characteristic in longstanding GAN.

Intracranial meningiomas in childhood and adolescence

PRIMARY TUMORS of the meninges are uncommon in childhood and adolescence. The incidence of meningiomas in most series of intracranial tumors of adulthood varies from 10 to 20%. However, Mendiratta et al.,1 in reviewing 15 series of intracranial tumors of childhood, found that 38 of 2,620 cases, or 1.5%, were meningiomas. Of their 38 patients, 2 were infants and the rest, older children. Cushing and Eisenhardt,? in their series of 315 patients with meningiomas, found only 6 (1.9%) who were less than 20 years of age.

Pediatric wegener’s granulomatosis complicated by central nervous system vasculitis

An unusual case of central nervous system vasculitis in pediatric Wegeners granulomatosis, a rare disorder that infrequently presents during childhood, is reported. A 13-year-old girl with Wegeners granulomatosis, whose initial presentation resembled Henoch-Schonlein purpura, developed recurring seizures. MRI of the brain demonstrated multiple areas of increased signal in the occipital, parietal, and frontal lobes, consistent with central nervous system vasculitis. Although both peripheral and cranial neuropathies have been reported in patients with Wegeners granulomatosis, cerebral vasculitis is unusual, particularly in childhood. This case emphasizes the need to consider Wegeners granulomatosis in the differential diagnosis of both unexplained seizures and central nervous system vasculitis in children with systemic illness.

Neuropathology of progressive rubella panencephalitis after childhood rubella

We studied neuropathologic, virologic, and immunobiologic aspects of a patient with acquired rubella developing progressive rubella panencephalitis. There were widespread white matter lesions with active vasculitis. Deposits of IgG were found in histologically normal cerebral vessels, and extracts of brain demonstrated large amounts of rubella-specific antibody. These studies suggest an immunologic component in this form of encephalitis.

β‐Adrenergic treatment of hyperkalemic periodic paralysis

In a patient with hyperkalemic periodic paralysis, metaproterenol prevented muscular weakness and hyperkalemia in periods of rest after exercise. During a severe attack, the drug rapidly corrected hyperkalemia and seemed to enhance the return of strength. The action of metaproterenol may involve a β-adrenergic-mediated increase of potassium transport via the sodium-potassium pump.

On national needs for neurologists in the United States

Estimates of the number of neurologists needed in the United States have been widely divergent. For direct patient care needs alone in a US population of 243.5 million, the Joint Commission on Neurology (ANA-AAN) estimate was 14,000 neurologists, the Delphi Panel median (Battelle/GMENAC) 11,200, and the Advisory Panel (Battelle/GMENAC) 6,200; our review suggests a need for 12,600. Total needs for clinical neurologists including faculty were 8,400 (Advisory Panel), 14,500 (Delphi Panel), and 16,500 (ours) for respective rates of 3.44, 5.96, and 6.76 neurologists per 100,000 population.

Ambulatory EEG recordings in epileptic and nonepileptic children

We examined the cassette-recorded 24-hour ambulatory EEG findings in children who had either clinically definite seizures or episodic behavioral disturbances not regarded as epileptic on clinical grounds. Among 40 epileptic patients, 22 had one or more attacks during the 24-hour recording session. In 15 of these patients all clinical attacks had appropriate ictal electrographic accompaniments; in another 6 some (but not all) attacks did so. Among 55 children with nonepileptic spells clinically, the 24-hour recording was uninterpretable for technical reasons in one, and in 30 it provided no relevant information because there were no recorded clinical or electrographic attacks. In the remaining 24 patients, one or more clinical attacks were captured, and in no instance was there any accompanying electrographic change. Our findings indicate that the absence of ictal EEG changes during attacks cannot be used in isolation to make a diagnosis of pseudoseizures, but support such a diagnosis made on clinical grounds. The more important role of the ambulatory EEG is to exclude a diagnosis of nonepileptic attacks by demonstrating electrographic seizure activity accompanying typical clinical attacks. Whether the technique will have a useful role in the evaluation of patients when the nature of an episodic disturbance of cerebral function is unclear clinically remains to be established, but will require long-term follow-up to validate any conclusions reached by the electrophysiologic technique.

Variability of serial CT scans in subacute necrotizing encephalomyelopathy (Leigh disease).

Computed tomographic (CT) brain scans of patients with subacute necrotizing encephalomyelopathy (SNE) may reveal focal lesions that correspond to sites of anatomic involvement of the disease. Three patients with SNE were followed with serial CT brain scans. In two patients radiographic abnormalities appeared well after the onset of clinical symptoms. In all three patients the radiographic lesions changed with time. This variability seen with serial scanning is an important feature of SNE.

A new familial arthrogryposis without weakness

A father and two daughters have a distinctive form of arthrogryposis multiplex congenita in which there is neither weakness nor atrophy about the affected joints despite the existence of myopathy. Contractures of the hips arid shoulders are present in all three individuals, but only the father has severe involvement of the hands and feet. Serum creatine phosphokinase values are 1.5 and three times normal in the daughters and five times normal in the father. Although it is likely that the myopathy and arthrogryposis are due to the effects of a dominantly inherited gene that appears as a new mutation in the father, the mechanism of production of the arthrogryposis in the absence of weakness is unknown.

Neurologists in the United States—past, present, and future

Neurologists in the United States were enumerated for each year from 1935 to 1984 on two bases: board certification (including Child Neurology and Psychiatry and Neurology) and completion of PG4 neurology residency training. The annual incidence of new neurologists was calculated at less than 200 until 1970; then it rose steadily to 380 in 1980, and plateaued thereafter at 385. The estimated number of neurologists present at one time (prevalence) was 1,500 in 1950, 2,400 in 1970, 4,600 in 1980, 8,100 in 1990, and 11,000 in 2000. These numbers for total neurologists will plateau at 12,200 by about 2010, at which time the number of certified neurologists will also plateau at 9,900. Both numbers are notably less than our prior estimate of needs: 16,500 neurologists by 1990, 19,100 in 2010.