Alan R. Seay

Cardiac arrest during induction of anesthesia in Duchenne muscular dystrophy.

I. McCracken GH, and Mize SG: A controlled study of intrathecal antibiotic therapy in gram-negative enteric meningitis of infancy: Report of the Neonatal Meningitis Cooperative Study Group, J PEtm,rR 89:66, 1976. 2. Gross RJ, Rowe B. and Easton JA: Neonatal meningitis caused by Citrobacter koseri, J Clin Pathol 26:138, 1973. 3. Gwynn CM, and George RH: Neonatal citrobacter meningitis. Arch Dis Child 48:455, 1973. 4. Tamborlane WV. and Soto EV: Citrobaeter diversus meningitii: A case report, Pediatrics 55:739, 1975. 5. ttarris D, and Cone TE: Escherichia freundii meningitis: Report of two cases, J PEt~tATg 56:774, 1960. 6. Sedlak J: Present knowledge and aspects of Citrobacter, Curr Top Microbiol lmmunol 62:41, 1973. 7. Jones SR, Ragsdale AR, Kutscher E, and Sanford JP: Clinical and bacteriologic observations on a recently recognized species of Enterobacteriaceae, Citrobacter diversus, J Infect Dis 128:563, 1973. 8. Madrazo A, Geiger J, and Lauter CB: Citrobacter diversus at Grace Hospital, Detroit, Michigan, Am J Med Sci 270:497, 1975. 9. Cussen LJ, and Ryan GB: Hemorrhagic cerebral necrosis in neonatal infants with cnterbacterial meningitis, J PEDIATR 71:771, 1967. 10. Shortland-Webb WR: Proteus and coliform meningoencephalitis in neonates, J Clin Pathol 21:422, 1968. I1. Rance CP, Roy TE, Donohue WL, Sepp A, Elder R, and Finlayson M: An epidemic of septicemia with meningitis and hemorrhagic encephalitis in premature infants, J PEDIATR 61:24, 1962. 12. Hoffman H J, Hendrick EB, and ttiscox JL: Cerebral abscesses in early infancy, J Neurosurg 33:172, 1970. 13. Eberhard S J: Diagnosis of brain abscesses in infants and children: A retrospective study of twenty-six cases. NC Med J 30:301, 1969. 14. Samson DS, and Clark K: A current review of brain abscess, Am J Med 54:201, 1973.

Rigid Spine Syndrome: A Type I Fiber Myopathy

The rigid spine syndrome is an unusual disorder characterized by nonprogressive mild axial and proximal muscle weakness, flexion contractures of the neck and spine, scoliosis, and joint contractures. The biopsy specimen from the patient described in this report shows type I fiber hypotrophy and predominance. This contrasts with previous reports that describe connective tissue proliferation as the major histologic abnormality.

Peripheral mononeuropathy complicating anaphylactoid purpura

5. Centers for Disease Control: Pneumocystis pneumonia among persons with Hemophilia A. MMWR 31:365, 1982. 6. Marx JL: New disease baffles medical community. Science 217:618, 1982. 7. Kornfeld H, Vande Stouwe RA, Lange M, Reddy MM, et al: T-lymphocyte subpopulations in homosexual men. N Engl J Med 307:729, 1982. 8. Ziegler JL, Miner RC, Rosenbaum E, Lennette ET, et al: Outbreak of Burkitts-like lymphoma in homosexual men. Lancet 2:631, 1982. 9. Lederman MM, Ratnoff OD, Scillian J J, Jones PK, et al: Impaired cell-mediated immunity in patients with classic hemophilia. N Engl J Med 308:79, 1983. 10. Menitove JE, Aster RH, Casper JT, Lauer SJ, et al: Tlymphocyte subpopulations in patients with classic hemophilia treated with cryoprecipitate and lyophilized concentrates. N Engl J Med 308:83, 1983.

CT scans in Menkes disease

The clinical courses and serial computerized tomography (CT) scans of four patients with Menkes disease are described. Although the initial clinical presentations were similar, head growth and serial CT scans showed striking individual differences. The CT scans varied from showing no abnormalities early in the disease to showing diffuse cortical atrophy, subdural accumulation of fluid, or multifocal areas of ischemic infarction. The pathologic findings in one patient showed only cerebral and cerebellar atrophy, whereas the findings in another patient showed areas of ischemic infarction, probably secondary to abnormal vessels. Menkes disease should be suspected in male infants with psychomotor deterioration and seizures, or when trauma is suspected from subdural hematoma and multiple fractures.

Pattern and concentration of IgG in cerebrospinal fluid in neurosarcoidosis

Fteports have suggested that the pattern of CSF IgG dif-ferentiates neurosarcoidosis from multiple sclerosis. We examined CSF and serum of 7 patients with neurosarcoidosis to determine concentrations of IgG and al-bumin and the presence of oligoclonal bands. Our results showed that neurosar-coidosis may have associated abnormalities of IgG synthesis and oligoclonal bands present in CSF, but without a consistent pattern.

Defective neutrophil function in myotonic dystrophy

Abstract Polymorphonuclear leukocytes (PMNs), obtained from 10 patients with myotonic dystrophy and 39 age-matched controls, were tested for chemotactic activity, chemiluminescence (CL), and phagocytosis. PMNs from 8 of the 10 patients had depressed chemotactic indexes (30 ± 23) as compared to controls (61 ± 18) (P

Serum creatine phoshokinase and pyruvate kinase in neuromuscular disorders and Duchenne dystrophy carries

Serum levels of creatine phosphokinase (CPK) and pyruvic kinase (PK) were determined on 42 controls, 57 patients with various neuromuscular disorders, and 23 female relatives of Duchenne dystrophy patients. CPK and PK enzyme activities were increased incomparable numbers of muscle disease patients and female carriers; there was no advantage in the combined use of the two tests. We did not confirm earlier reports suggesting that determination of PK is more valuable than CPK in carrier detection.

Interferon treatment of experimental Ross River virus polymyositis

Ross River virus (RRV), an alpha togavirus, causes an inflammatory myopathy in mice, which probably results from direct lytic effects of virus or viral products on myofibers. Administration of recombinant hybrid human leukocyte interferon-alpha A/D (rINF-αA/D) ameliorates clinical illness and reduces mortality from 86 to 42%. Peak concentrations of virus are reduced by 1,000-fold in serum and by 30-fold in muscle, but anti-RRV antibody production is not altered. Treatment with rIFN-αA/D dramatically reduces inflammation and necrosis in muscle. Beneficial effects of rIFN-αA/D on experimental, RRV-induced polymyositis result in part from inhibition of viral replication and spread, though immunomodulation might also play an important role.

1154 MENKES KINKY HAIR SYNDROME: SERIAL CT SCAN FINDINGS IN THREE CASES WITH PATHOLOGICAL CORRELATION IN ONE CASE

The clinical courses and C-T scans of 3 patients with Menkes Kinky Hair Syndrome (MKHS) are described. Once the diagnosis was considered and confirmatory evidence of disturbed copper metabolism was collected the overall clinical neurological courses were similar. However, striking variation was noted in the patterns of head growth, varying from early and consistent microcephaly to accelerated head growth and increased intra-cranial pressure. Similar variation in the C-T scan data was noted, from diffuse cerebral atrophy to cystic encephalomalacia to findings which suggested large bilateral subdural hematomas. An angiogram and autopsy in one case document respectively striking blood vessel abnormalities and old infarcts and ischemic lesions. These case findings illustrate the need to consider MKHS in young infants with retarded growth, delayed development and seizures in whom other clinical and radiographic evidence strongly suggests abuse and neglect.