Ramon Manon-Espaillat

Antiepileptic drug intoxication: factors and their significance

SUMMARY: A retrospective chart review (1979–1985) was performed to identify probable causes of intoxication with antiepileptic drugs (AEDs). We identified 141 patients meeting clinical and laboratory criteria for intoxication and 17 with clinical signs but with serum concentrations within the therapeutic range. The majority were epileptic patients; almost half were treated with monotherapy, most with phenytoin (PHT). The causes of intoxication in the epileptic patients were iatrogenic (41%), inappropriate dose self‐adjustment (34%), suicide attempt (18%), inappropriate caretaker dose adjustment (9%), accidental ingestion (8%), unrecognized drug interaction (6%), and association with intercurrent illness (2%). Twenty‐two patients had more than one probable cause of intoxication. In nonepileptic patients the causes were suicide attempt (50%), accidental ingestion (27%), and iatrogenic (23%). Most patients had signs of ocularmotor and vestibulocerebellar dysfunction. Rarely described manifestations of intoxication, such as seizures or choreoathethosis, were observed in a few patients. The average hospital stay was 6.9 days; there was no mortality, and all patients recovered fully. We conclude that AED intoxication is a major but preventable cause of morbidity and that suicide attempts are an important and underrecognized contributor in both epileptic and non‐epileptic patients.

Familial 'sleep apnea plus' syndrome: Report of a family

We describe a familial disorder consisting of sleep apnea, anosmia, colorblindness, partial complex seizures, and cognitive dysfunction. The phenotypic expression of the syndrome suggests an autosomal dominant inheritance with incomplete penetrance.

Dissociated weakness of sternocleidomastoid and trapezius muscles with lesions in the CNS

We describe three patients with lesions in the CNS that produced dissociated weakness of trapezius (TM) and sternocleidomastoid muscles (SCM). One patient with a right cerebral hemisphere lesion had left TM weakness and right SCM weakness. A second, with a brainstem lesion, and a third, with a high cervical cord lesion, had TM weakness with preserved SCM strength. We discuss the findings according to the known neuroanatomy.

Sleep Apnea in Multiple Sclerosis

We performed polysomnography on eight randomly chosen male patients who met Posers criteria for definite multiple sclerosis (MS), to obtain an idea of the frequency and severity of sleep apnea (SA) in this population. Ages ranged from 27 to 67 years (mean, 50 years). The mean Kurtzkes disability score was 6.1. Only one complained of awakenings with shortness of breath. Otherwise, none had a history of sleep disorder or significant pulmonary disease. We found that two patients had an apnea index greater than 5, with oxygen desaturation to 60% in one. Two others had apnea indices less than 5, but had oxygen desaturation to 59% and 81% during the apneas. Two patients had apnea indices less than 5 without oxygen desaturation. The apneas were mainly central type, except in one who had a mixed central-obstructive pattern. These preliminary results suggest that SA in MS may be more frequent than suspected, and it can be associated to significant oxygen desaturation in some cases. The potential impact of SA in MS deserves further evaluation. Key Words: Sleep apnea—Multiple sclerosis—Automatic respiration—Central apneas—Apneas with central nervous system lesions.

Sternocleidomastoid and trapezius dissociation

analyzed 3 caws of patienta with weakness of muscles supplied by the accessory nerve. Their caee 1 was a patient who had left hemipareek due to an internal capsule lesion. They reported that the patient had the expected weakneee of the left TM, but the SCM waa weak on the right. The explanation prwided is that the corticoepinal pathway to the SCM (but presumably not to the TM) has a double decuseation in the brahatem. It is unclear how this could explain the diaeocieted wealmeee of the SCM and TM with a lesion above the first decuseation. Although in general each cerebral hemisphere controls the motor activity of the contralateral side of the body, there are exceptions to this rule, the most widely known of which are the uppermost branches of the facial nerves, which allegedly have bilateral cortical representation. A similar situation o c c m with regard to the diaphragm, although here the cortical representation is not symmetric bilaterally. Unilateral hemiplegia does not include diaphragmatic paralysis. but still the diaphragm on the involved side is less active.6.7 As opposed to the view of Manon-Espaillat and Ruff of double decussation, Balngura and Katze claimed that the supranuclear innervation of the SCM is undecuesaW, both views apparently further violate the rule of contralaterality. However, there are alternative interpretations to the resulta of Manon-Espaillat and Rufp and of Balagura and Katz.8 In both studies, the investigators examined subjects with hemiplegia and compared the power of their SCM on either side. While they did not specify exactly how the test wae carried out, one is left with the impression that they examined head turning to the opposite side. However, these muscles have additional actions, namely tilting the head toward the ipsilateral shoulder, as well as-when both muscles act together-neck flexion. We have carefully examined 12 patienta with hemiplegia (age 63 to 79 years; 3 women; 7 with right hemisphere lesion). We can confirm the previous findinga that head turning toward the involved side waa weak in all, although the weakness was never extreme. However, when they were ordered to flex their necks against resistance, the SCM was weak ipsilaterally to the paralyzed side, thus causing a tilt of the head with the chin toward the same side. When ordered to try to touch their shoulders with their ears against resistance, all patienta again had obvious weaknew on the side contralateral to the hemispheric lesion. Thus, although all pntienta had weahem of both SCMe, this weakness was prominent on the side ipeilated to the brain h i o n when head turning toward the opposite side was teat%& but on the contralateral side when other functions of the eame muecle were examined Each cerebral hemisphere is responsible not only for the motor control of the opposite side of the body, but for dealing with the contralateral side of the external world in general. This is clearly Been in the cortical representation of the visual fields. Similarly, each frontal lobe controls voluntary gaze of both eyes toward the opposite side. Head turning is intimately connected with gaze, and it is therefore not surprising to UB that with unilateral hemispheric lesions, ocular movementa as well ae head turning are weak toward the contralateral side. This resulta in the “paradoxic” impression thnt the innervation of the SCM is contralateral for he& turning, but ipsilateral for other functions of that muscle. Therefore, both ipsilateral and contralateral control must be exerted by the cerebral cortex on spinal motor neurons of the SCM.

Tolerance to Beneficial and Adverse Effects of Antiepileptic Drugs

I am very pleased with the second edition of this practical manual of neuro-ophthalmology newly revised by Lanning Kline, MD, with contributions from Patrick S. O’Connor, MD. The first edition, solely written by Dr. Frank Bajandas, appeared in 1980 and was aimed at physicians training in the clinical neuroaciences who were preparing for specialty board exams. Since then, the book has developed a wider reputation as a synopsis of useful approaches to everyday problems encountered in clinical neuro-ophthalmology. Tragically, Frank Bajandas died an untimely death, but I am sure he would have agreed with me that the majority of changes made to the second edition of his book are enhancements rather than detractions. The book is now sold only in hard cover which, combined with inflation, probably explains the increase in price. (My two softbound copies of the first edition are literally falling apart from frequent use by me and others.) Three new chapters have been added covering cavernous sinus syndromes, ocular myasthenia and related disorders, and ancillary procedures (temporal artery biopsy, Tensilon test, confrontation visual field testing, and forced duction testing), The chapter in the first edition that dealt with the fifth nerve and headache has now been suitably split into two separate chapters. The present edition is 35 pages longer than the first and now contains an index. The references have been appropriately updated. The text remains in outline form and numerous diagrams and tables are present, often representing additions and deletions to those in the first edition. Some of these changes are improvements, but others are not. For example, the addition of drawings of a patient’s eyes to the “wiring diagrams” depicting an internuclear ophthalmoplegia and a “one and a half’ syndrome is confusing, since only one horizontal gaze position is shown in each case. Also, the revised diagram of the ParksBielschowsky three step test for diagnosis of cyclovertical muscle paresis is more confusing than the one in the first edition. However, the diagrams that supplement the new chapter on the pupil are significant improvements, as are those chosen for the revised chapter on the fourth cranial nerve. These criticisms are small points, however, and should not negatively influence the prospective buyer. The first edition was dedicated to three contemporary giants in neuro-ophthalmology (J. Lawton Smith, Joel Glaser, and Robert DarofT) and their presence continues to be felt in this revision. I highly recommend it to those who need a lucid and concise review of clinical neuro-ophthalmology.