dure can be performed easily because patients in question would be maintained on respiratory support. Neuromus- cular blocking agents are devoid of central nervous system effects [GI.

EKG contamination is so common in high-sensitivity EEG recordings that its absence should alert the tech- nologist to examine the integrity of the recording system. Baseline irregularity is often exaggerated by ballisto- cardiographic sffects that are identifiable by a regular re- lationship to the QRS complex of the EKG.

At the sensitivity employed, electrical noises generated within the amplifiers may be substantial. They are often unevenly distributed between EEG channels, and adequate calibrations should be provided. Further, electromagnetic field characteristics are highly variable and unstable at set- tings used in portable EEG recording and can contribute both intermittent and continuous wave activity resembling cerebral electrical potentials.

EEG Laboruzory Department of Neurology University of Utah College of Medicine Salt Luke City, U T 84132

References 1. American Electroencephalographic Society: Minimum techni-

cal standards for EEG recording in suspected cerebral death. In Klass DW, Daly DD (eds): Current Practice of Clinical Elec- troencephalography. New York, Raven, 1979, pp 492-496

2. American Neurological Association: Statement regarding method for determining that the brain is dead. Trans Am Neurol Assoc 101:322-323, 1976

3. Ashwal S, Schneider S: Failure of electroencephalography to diagnose brain death in comatose children. Ann Neurol

4. Knott JR: Identification of EKG-related artifacts in very low

5. Radberg C, Soderlundh S: Computer tomography in cerebral

6. Toman JE, Davis JP: The effects of drugs upon the electrical

6512-517, 1979

voltage EEG records. Am J EEG Techno1 16:129-130, 1976

death. Acta Radio1 [Suppl] (Stock) 346: 119-129, 1975

activity of the brain. Pharmacol Rev 1:425-492, 1949

Brief Treatment with Dichloroacetate Does Not Modify Suspected Subacute Necrotizing Encephalomyelitis Owen B. Evans, MD, and Peter W. Stacpoole, MD, PhD

Subacute necrotizing encephalomyelitis (SNE) is a neuro- pathological diagnosis often associated with disordered pyruvate metabolism. Inhibition of thiamine triphosphate synthesis [4] and, recently, defective regulation of the pyruvate dehydrogenase complex (PDHC) 131 have been reported in patients with SNE. DeVivo et a1 [31 suggested the use of dichloroacetate (DCA) in treatment of these disorders. We report a brief trial of DCA in a child with suspected SNE.

A 51/2-year-old girl had presented at 11/2 years with ataxia and tremor. She subsequently developed progres- sive ataxia, dysmetria, ophthalmoplegia, bulbar paresis, polyneuropathy, growth retardation, persistent tachycar- dia, episodic fever, and hypertension. Laboratory studies showed mildly elevated urine catecholamines and hy- perlactatemia that was exacerbated by a glucose chal- lenge. Inhibition factor for thiamine pyrophosphate- adenosine triphosphate phosphotransferase was demon- strated in the urine (courtesy of Dr J. R. Cooper).

Biochemical studies showed normal PDHC, pyruvate decarboxylase, a-ketoglutarate dehydrogenase, and lipo- amide dehydrogenase activity in muscle, fibroblasts, and platelet-enriched plasma. Muscle histochemistry study was normal for cytochrome c oxidase. Modifying the platelet P D H C assay [l] by using 10 mM phosphate buffer and shortening the incubation period to 5 minutes showed activation of PDHC following preincubation with 20 mhf magnesium chloride and 2 mM calcium chloride. The pa- tient showed reduced basal P D H C activity (99 pmol/ minimg protein) compared to 5 healthy controls (221 * 93.1) but higher activated PDHC (357 versus 278 * 52). The value of (PDHC activated - P D H C basal)/(PDHC basal) x 100 [3] was 26196, compared with 120 * 38% in controls; this difference was not statistically significant. Because platelet isolation favors activation, the finding of low basal PDHC activity in isolated platelet-enriched plasma preparations is similar to the abnormalities of PDHC regulation in the child reported by DeVivo et al [3]. The relationship of thiamine triphosphate synthesis inhibi- tion to possible defective PDHC regulation is not known.

When the patient was 3 years old, before her biochemi- cal defect had been identified and before the discovery of chronic D C A neurotoxicity [ 5 ] , she was hospitalized for a therapeutic trial of DCA for hyperlactatemia. Informed consent was obtained from the child’s parents. The drug was given as a single daily oral dose. The mean blood lac- tate level 10 days before treatment was 3.46 mMol per liter t 29 SEM (normal, <2.10). During the continuous 10-day

Notes and Letters 647

treatment period, blood lactate decreased to 2.64 & 0.22 on a DCA regimen of 20 mg/kg/day for 7 days and to 1.90 & 0.10 with 30 mg/kg/day for 3 days ( p < 0.05). The child showed no complication or clinical improvement during drug therapy.

Ours is the first report of D C A in the treatment of a PDHC disorder. This and other studies 121 have shown the efficacy of DCA in treating hyperlactatemia. However, be- cause of the complexjty of P D H C regulation, hyperlac- tatemia may result from other biochemical disturbances which secondarily affect P D H C activation. Therefore, re- duction of blood lactate per se may not affect the primary disease process. Longer treatment periods would be neces- sary to determine if DCA can arrest the progression of SNE. Animal studies are under way to study the cause of DCA neurotoxicity and to investigate less toxic analogues of therapeutic potential. At present, chronic administration of the drug cannot be recommended.

Supported in part by agrant from the Muscular Dystrophy Associ- ation.

Department of Neurology and Division of Endocrinology,

Vanderbih University Medical Center Nashville. T N 3 7232

References

Department of Medicine

1. Blass JP, Cedarbaum SD, Kark RAP: Rapid diagnosis of pyru- vate and keroglutarate dehydrogenase deficiencies in platelet- enriched preparations from blood. Clin Chim Acta 75:2 1-30, 1976

2. Coude FX, Saudubray JM, DeMaugie F, et al: Dichloroacetate as treatment for congenital lactic acidosis. N Engl J Med 299:1365-1366, 1978

3. DeVivo DC, Haymond M W , Obert KA, et al: Defective acti- vation of the pyruvate dehydrogenase complex in subacute nec- rotizing encephalomyelopathy (Leigh disease). Ann Neurol

4. Pincus JH: Subacute necrotizing encephalomyelopathy (Leigh’s disease): a consideration of clinical features and etiology. Dev Med Child Neurol 14:87-101, 1972

5. Stacpoole PW, Moore GW, Kornhauser DM: Toxicity of chronic dichloroacetate. N Engl J Med 300:392, 1979

6:483-494, 1979

Failure to Include Coauthor of Paper Hugo W. Mnser, M D

Due to an oversight on my part, D r Aubrey Milunsky’s name failed to be included in the paper entitled “Ad- renoleukodystrophy : elevated C-26 fatty acid in cultured skin fibroblasts,” by Moser HW, Moser AB, Kawamura N , Murphy J, Milunsky A, Suzuki K, Schaumburg H, Ki- shimoto Y (Ann Neurol 7:542, 1980). That this was in- deed an oversight is evidenced by the fact that the Eunice Kennedy Shriver Center had been listed as one of the places of origin, and none of the other authors is associated with that center. D r Milunsky’s name has been added to all the reprints of the paper. I take full responsibility for chis unintended oversight and apologize for it.

The John F . Kennedy Initiiute 707 hT Broadway Baltimore, M D 21205

648 Annals of Neurology Vol 8 No 6 December 1980