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Electromyography in myotonic dystrophy.

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extrinsic spread from contiguous nerves. A similar mechanism of spread to the ophthalmic and central retinal arteries
via the nasociliary nerve may be implicated in our patient.
Antidromic spread of HZ angiitis was recently suggested
by MacKenzie e t al [4].Noting that the ophthalmic nerve
innervates the dura, venous sinuses, large cerebral veins,
and arteries at the base of the brain, they suggested spread
of varicella zoster virus along the intracranial branches of
the ophthalmic nerve. Such a mechanism might explain the
well-described arteritis of the carotid, middle cerebral, and
anterior cerebral arteries seen ipsilateral to HZ ophthalmicus. Similarly, the proximity of the ophthalmic nerveinnervated dura to the middle meningeal artery might explain its involvement in our patient. Oculosympathetic
paralysis is a rare complication of HZ infection and has
been ascribed to sympathetic interruption at the middle ear
[2] or internal carotid artery [5].In our case, it seems more
likely that the paralysis resulted from antidromic spread of
varicella zoster virus to the long ciliary nerve.
Department of Neuvology
Bowman Gray School of Medicine
Winston-Salem. NC 271 03
1. Hollenhorst RW: Symposium on Neuro-ophthalmology. St.
Louis, Mosby, 1976, chap 13, pp 168-190
2. Jarrett WH: Horner’s syndrome with geniculate zoster. Am J
Ophthalmol 63:326-329, 1967
3. Linneman CC Jr, Alvira MM: Pathogenesis of varicella-zoster
angiitis in the CNS. Arch Neurol 37:239-240, 1980
4. MacKenzie RA, Forbes GS, Karnes WE: Angiographic findings
in herpes zoster arteritis. Ann Neurol 10:458-464, 1981
5. Victor DI, Green WR: Temporal artery biopsy in herpes zoster
ophthalmicus with delayed arteriris. Am J Ophthalmol
82:628-630, 1976
social skills of the 10- to 11-months’ level. The patient had
a seizure disorder with a right temporal lobe epileptic focus
controlled by primidone, 50 mg twice daily. Examination of
cranial nerves and of the sensory and motor systems
showed no abnormalities.
In 1976, the patient became progressively hyperactive in
spite of therapy with chlorpromazine, 75 mg twice a day.
Results of general physical and neurological examination
and laboratory work were unchanged. Primidone was discontinued and the patient was given phenytoin, 50 mg
twice daily, and diazepam, 5 mg three times per day.
Thioridazine, 15 mg three times daily, was given from
March, 1977, to March, 1978, without improvement. From
March, 1978, to October, 1979, methylphenidate at a dosage of 10 mg daily was tried without success.
I n November, 1979, magnesium pemoline, 37.5 mg per
day, was started and increased to 7 5 mg daily after five
weeks, resulting in an improvement in behavior. In July,
1980, the patient was noted to have violent choreiform involuntary movements of all the extremities and dyskinetic
movements of the face and tongue. Other findings were
unchanged. Pemoline was discontinued, and the abnormal
movements slowly diminished and completely disappeared
over the next 48 hours. At present, the patient is taking
phenytoin alone and is slowly becoming hyperactive.
Experiments in guinea pigs suggest that pemoline increases central dopaminergic activity, but the exact mode
of action is unknown. High doses of amphetamine produce
psychosis as well as abnormal choreiform movements due
to indirect stimulation of dopaminergic receptors. Prolonged use of amphetamines produces hypersensitivity of
the dopaminergic neurons in the basal ganglia [2]. A similar
effect may explain the chorea observed in this child after
therapeutic doses of magnesium pemoline.
New York Medical College
Mental Retardation Institute
Westcbester County Medical Center
Valhalla, NY 10595
Chorea in Long-term
Use of Pemoline
Baldev Kaur Singh, MD, Avtar Singh, MD,
and Emanuel Chusid, MD
Magnesium pemoline has been used to treat hyperactivity
in children because of its central stimulant action, which is
comparable to the effect of dextroamphetamine [ 11.
Chorea has been reported in a child suffering from
pemoline overdose 131 but not previously following
therapy in the usual dosage.
A 9-year-old boy with normal prenatal and perinatal
history developed hyperactive behavior after the age of 1
year. He stood at 2 years and spoke and walked at age 3. In
1975, at the age of 6 years, he was initially treated because
of uncontrolled hyperactivity and psychomotor retardation. H e had received chlorpromatine and diazepam for his
behavior. General physical examination was normal. The
head circumference was average; height and weight were in
the second percentile. He was hyperactive, had a short attention span, performed up to the level of 22 months in
motor coordination, and possessed adaptive, verbal, and
218 Annals of Neurology
Vol 1 3 No 2
February 1983
1. Conners CK, Taylor E, Meo G, Kurrz MA, Fumier M: Magnesium pemoline and dextroamphetamine: a controlled study
in children with minimal brain dysfuncrion. Psychopharmacologia 26:32 1-336, 1972
2. Klawans HL, Margolin DL: Amphetamine induced dopaminergic hypersensitivity in guinea pigs. Arch Gen Psychiatry
32:725-732, 1975
3. Nausieda PA, Koller WC, Weiner WJ, Klawans HL: Pemolineinduced chorea. Neurology ( N Y ) 31:356-360, 1981
Electromyography in
Myotonic Dystrophy
Erich Streib, MD, and Sallie F. Sun, MD
Drs Pryse-Phillips, Johnson, and Larsen, the authors of a
study of a large kinship with myotonic dystrophy (MyD) recently published in the Annals, should be congratulated for
their thorough investigation in a patient group which is usually not very cooperative [3]. In their study, subjects with
unequivocal myotonia by clinical or electromyographic
(EMG) criteria were categorized as affected with MyD, subjects without myotonia as having a “partial syndrome” of
Bundey et al {l] and Polgar et al{2] found EMG evidence
of myotonia in all their subjects with clinical features of MyD.
Moreover, myotonia was present on the EMG in a few subjects with normal clinical examinations. It has been shown
that EMG-demonstrated myotonia may be restricted to individual muscles in MyD {47. In our own studies, EMG evidence of myotonia was present in all examined muscles in
only 3 of 25 subjects with clinical MyD. Myotonia was most
common in distal limb and facial muscles, being positive in
the latter in 21 of 22 subjects examined.
Unfortunately, the authors do not state which muscles and
how many were examined electromyographically. It is possible that study of several limb muscles and the facial muscles
may have documented myotonia by EMG in more cases,
since 12 of 27 patients with the “partial syndrome ” had facial
weakness as indicated in their Table 3.
Department of Neurology
University of Nebraska College of Medicine
Omaha. N E 68105
Bundey S, Carter CO, Soothill J F Early recognition of heterozygotes for the gene of dystrophia myotonica. J Neurol Neurosurg
Psychiatry 33:279-293, 1970
Polgar JG, Bradley WG, Lipton ARM, Anderson J, Howat JML,
Petito F, Roberts DF, Scopa J: The early detection of dystrophia
myotonia. Brain 95:761-766, 1972
Pryse-Phillips W, Johnson GJ, Larsen B: Incomplete manifestations of myotonic dystrophy in a large kinship in Labrador. Ann
Neurol 11:582-591, 1982
Rosselle N, Stevens A, Sonck W: An electromyographic study of
multiple cases of myotonia atrophica in one same family. Electromyogr Clin Neurophysiol 10:415-424, 1970
William Pryse-Phillips, MD, FRCP
Dr Streib and Dr Sun make a valid point, for which we
thank them. Among patients with MyD in our series, myotonic runs were detected on the EMG in all, since this was a
diagnostic criterion. We found that clinical and EMG myotonia were perfectly correlated in our MyD subjects; myotonic runs were detected in all of 3 1 abductor pollicis brevis,
14 of 19 biceps, 4 of 4 brachioradialis, and 5 of 8 flexor carpi
radialis, trapezius, deltoid, infraspinatus, or tibialis anterior
examinations. In all, we examined 62 muscles in 32 patients.
After a short time we developed so much confidence in the
results of EMG study of the abductor pollicis brevis that only
curiosity led us to sample other muscles.
The partial syndrome was identified only after examination
of the data at the end of the study. Among the 26 patients in
whom EMG was done, we sampled 43 muscles, including the
abductor pollicis brevis in all and the biceps, brachioradialis,
deltoid, extensor carpi radialis longus, abductor digiti minimi,
or extensor digitorum brevis in most. While 4 subjects
showed abnormality, we did not, by definition, find myotonic
runs in any.
It is well accepted that distal muscles are more likely to
show myotonia than proximal muscles, and one may reasonably include the facial musculature in the former category.
Drs Streib and Sun found positive myotonic features in 2 1 of
22 facial muscles examined, and we found them in all 31
abductor pollicis brevis muscles of clinically affected patients.
Probably, the more distal the muscles sampled, the higher the
eventual yield, but we did not go further since we were already bordering on the limits of tolerance of our study population. In retrospect, it might well have been useful to have
examined facial muscles in addition to the other distal muscles sampled, since this might have moved a few patients
from the partial syndrome group into the MyD group; the
major conclusion of our paper, namely, that even minimal
abnormalities occurring in members of a MyD kinship hold
serious prognostic significance, would thus have been
strengthened further.
Division of Neurology
Health Sciences Complex
St. John’s, Njld, Canada AlB 3V6
Oph thalmoplegic
Polyneuropathy Associated
with Infectious
Abelardo Salazar, MD, Hector Martinez, MD,
and Julio Sotelo, MD
The syndrome of acute ophthalmoplegia, ataxia, and
areflexia is a variant of acute idiopathic polyneuritis that
commonly develops after a viral infection of the upper respiratory or gastrointestinal tract [31. We report a 34-yearold woman with this syndrome associated with infectious
mononucleosis (IM). To our knowledge only one similar
case has been reported, in a 31/-year-old boy [71.
On June 10, 1981, a 34-year-old woman was admitted to
the Instituto Nacional de Neurologia de Mkxico with
ophthalmoplegia and ataxia. O n June 1 she had presented
with hyperthermia, pharyngeal pain, headache, and nausea,
and was given penicillin and aspirin. Five days later, gait
instability, vertical diplopia, and left palpebral ptosis developed. The next day she could not move her eyes, gait
was ataxic, and she developed bilateral facial palsy. O n the
day of admission to the hospital she had all the referred
symptoms just described but she was afebrile. Physical
examination showed cervical adenomegaly, pharyngeal
hyperemia, and splenomegaly. Neurological examination
revealed complete bilateral external ophthalmoplegia with
mild bilateral ptosis, normal pupillary reflexes, bilateral
complete facial palsy, absent deep tendon reflexes, bilateral
dysmetria, marked ataxia, and mild hypotonia. The plantar
Notes and Letters 219
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electromyography, myotonic, dystrophy
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