Focal seizures induced by movement without sensory feedback mechanisms

403

Electroencephalography and Clinical Neurophysiology, 19741, 3 6 : 4 0 3 4 0 8 t ~ Elsevier Scientific Publishing Corapan~'. Amsterdam - Printed in The Netherlands

FOCAL

SEIZURES

INDUCED

BY MOVEMENT

WITHOUT

SENSORY FEEDBACK MECHANISMSI A. J. GAnOR Department of Neurol¢~ly, School ol" Medicine. Unirersity t!f Calilbrnia. Daris. Calil~ 95616 ¢t r.S..-I ) (Accepted for publication: October 24. 1973)

In cases where muscular activity provokes seizures, concomitant peripherally originating sensory information has been invoked as the "'trigger" for the clinical seizures. The evidence for such a mechanism is based upon the temporal relationship between the voluntary or involuntary muscular activity and the ensuing clinical state of the patients. Most commonly the seizures are precipitated by sudden movements, are generally tonic in character and have poor or nonexistent electrographic correlates to the seizure, Localization is difficult to determine except in rare instances (Lishman et al. 1962: Falconer et aL 1963: Whitty et aL 1964). The present case is reported becau~ the epileptic character of the disorder is clearly documented both clinically and electrographically and a unique and exceptional combination of clinical factors permitted examination of peripheral sensory elements as they relate to the genesis of such seizures.

polyuria. She had no previous history of a seizure disorder or head trauma. in the past the patient consumed large quantities of beer and 6 months prior to admission she was treated for alcoholic hepatitis confirmed by liver biopsy. Since that time she had refrained from heavy alcohol ingestion. General physical examination revealed the patient to be mildly stuporous but easily arousable. She had a normal pulse, blood pressure and respirations. Examination of the heart and lungs was normal, the liver was firm. nontender and not enlarged. Testing of cranial nerve function revealed only a mild left lower facial weakness. The deep tendon reflexes were variably ~-qual bilaterally or slightly hyperactive in the left upper extremity and there was a variable mild global weakness of that limb. Fine movements and rapid movements of the fingers of the left hand were carried out poorly. Reflexes and strength were normal elsewhere and the plantar reflexes w e r e flexor. S e n s o r y e x a m i n a l i t m at lime~ r e ~ e a l -

CASE PRESENTATION

A 49-year-old, right-handed female had been in good health until 6 days prior to admission to the Sacramento Medical Center. She developed progressive, generalized weakness and lethargy with episodes of left-side'*carpopedai spasm and facial distortion lasting 10-20 sec and occurring 20-50 times per da3 During these episodes she never lost consciousness, could always follow verbal commands and she was never incontinent. Over the previous 6 months she had a 30 pound weight loss, associated with polydipsia and t Presented in part at the 27th a:,nual meeting of the American Electroencephaiographic Society. June 1973.

ed decreased appreciation of position sense and 2 point discrimination in the left hand. During the course of several examinations. the patient could produce episodes ofcarpopedal spasm on the left without associated loss of consciousness by repeated grasping movements of the left hand. Following these episodes weakness of the left side of the face and left arm became more prominent. Laboratory examinations during her hospitalization revealed an anemia with a hemoglobin of 10 g, a normal white count, a serum sodium of 126 meq/I, potassium 3.8 meq/I, CO., 22 meq/I and a chloride of 93 meq/I. Serum glucose was 525 mg'~o and the urine revealed a marked

404

glucosuria and + I ketones with a serum pH of 7.46. Serum calcium was 9.2 mg°,o, SGOT was 65 units per ml, alkaline phosphotase 100 units and LDH was normal. Skull films, echo and brain scan were normal. A lumbar puncture revealed normal pressure and protein with no cells. CSF glucose was _. 9~1 m-"/ g ,,. An EEG showed interictal as well as ictal epileptiform discharges well localized to the right centro-parietal region. During her hospitalization she was treated with insulin and phenobarbital, initially 180 mg/day and later reduced to 120 mg/day, terminating the seizures. Discharge diagnosis was non-ketotic hyperglycemia and since her hospitalization she has been free of seizures and her diabetes is well-controlled. A detailed clirfical-electrographic correlation was carried out during her hospitalization with the aid of a 16-channel Grass model 6 electroencephalograph and 21 gold disc electrodes applied to the scalp with collodion according to the international placement system. A cinematographic record of the seizures was correlated with the concomitant e!ectrographic changes.

A . J . GABOR

sensory stimulation of the left hand were also ineffective. Most of the patient's seizures were heralded by a subjective sensation that the left hand was

1

RESUI.TS

All of the patient's clinical seizures were stereotyped. They occurred spontaneously but could also be induced by repeated flexion-extension movements of the left hand. There svas an irreducible refractory period. This refractory period was approximately 10-20 min in duration, during which time no seizure would occur spontaneously nor could a seizure be produced by any form of stimulation. The frequency of the patient's spontaneous seizures varied from day to day during her hospitalization and prior to anticonvulsant and insulin therapy ranged in fi'equency from one ,,-very 20 min to one every 12 h. Durh~g the time that the spontaneous seizures were relatively infrequent, repeated flexion and extension movements of the fingers of the left hand {grasping) produced seizures within 30-90 sec of initiating such movements. Similar movements of the right hand, passive movements of the left or right extremities, single sudden movements of the left hand, hyperventilation and photic stimulation were totally ineffective in initi~lting seizures. Various forms of repeated

C: Fig. I, Individual frames chosen from a film sequence corresponding to EEGs in Figs. 2-4 respectively. ,4:93 sec after patient began voluntary grasping movements of left hand, Patient experiencing sensation of movement. No movement is discernible. B: Early tonic stage with tonic elevation of arm and recruitment of left facial musculature. t ": Late clonic stage just prior to cessation of seizure and , u bsequent focal weakness.

405

FOCAL SEIZURES INDUCED BY MO~qEMENT

moving, "'wanted to move" or "'was going to move". During this sensation, however, no muscular activity could be discerned. This sensation or aura was associated with electrographic rhythmic low voltage slow waves at 3-4 c/see in the right hemisphere and well-restricted to electrodes C4 and 1)4 (Fig. l, A and 2). After a variable interval following this sensation, most commonly 2-3 sec, the patient developed extension of the fingers and flexion of the wrist. This was followed sequentially by tonic extension of the elbow and subsequent abduction of the shoulder and elevation of the left arm. Shortly thereafter the patient's head and eyes turned to the left and she developed tonic contraction of the left side of the face. This tonic activity was manifest electrographically by rhythmic low voltage spikes in the right central and parietal areas at a frequency of approximately 12/see. The spiking which continued to be maximal in this area was, however, reflected in homologous areas on the left, but at a considerably lower voltage {Fig. I, B and 3 ). The tonic muscular activity persisted for approximately 10~20 sec and merged slowly into clomc contractions of the left upper extremity and face and terminated abruptly. High voltage sharp waves and spikes at approximately 5 c/see were associated with the clonic phase of the seizure and the termination of the seizure was marked by an abrupt attenuation of electrical activity. primarily in the right hemisphere (Fig. 1. C and 4). Throughout the entire episode the patient was able to follow commands and indeed had absolute control over her eve movements. While the eyes did deviate to the left during version of the head to the left, she could be persuaded to carry out relatively normal right lateral gaze occasionally interrupted by nystagmus to the left. Clinical seizure activity was never observed in the lower extremity. Neurological examination immediately after the seizure revealed a marked weakness involving the left arm and face. The weakness was mainly manifest in the distal portion of the left arm and while the patient was able to elevate her arm at the shoulder against gravity, she was unable to elevate her wrist against gravity. The following day the patient underwent local

anesthetic block of the left brachial plexus. A total of 30 ml of0.7"o i, . .-,t u o c a l•n e w a s u s e d O v e r a period of 30 min marked vasodilation, a complete sensory anesthesia and motor paralysis in the domain of C5 through TI was produced. At this time the patient was requested to make repeated attempts to move the left hand in spite of the paralysis. The patient had not had any seizures during the day that this procedure was carried out. but within 90 sec of initiating attempts at movement the patient developed an electrographic pattern identical to the one associated with her clinical symptoms {Fig. 5). Prior to the seizure the EEG was indistinguishable from the interictal record obtained during the interictal period without anesthesia. Clinically. she manifested only contraversion of the head and eyes. At no time during the sei:,,~re was any movement noted in the left extremities. The patient recovered from the axiilary block within 6h. I)ISCUSSI()N

This case has been described primarily because it provides cause for speculation concerning thc origin of possible "reflex" mechanisms of movemcnt induced seizures. That some peripheral sensory mechanism, perhaps t'rom muscles or joints, provides the trigger for movementprovoked seizures is suggested in some reports (Alajouanine and Gastaut 1955. 1958: Gastaut and Tassinari 1966: Stevens 1966). In the present case. a brachial plexus block was produced to test this notit,,1. The primary assumption required, if one is to accept the results of such a test, is that the local anesthetic blocked all the crucial seizure provoking peripheral sensory impulses. The completeness of the anesthesia within the domain o." the area of altered sensation could be tested directly and is based upon the total loss of all sensation {including position, light touch, 2 point discrimination, deep pressure and pain sensation), motor paralysis and vasodilation. It may theretbre be assumed that all peripheral nerves irrespective of size subserving the dermatome pattern C5-TI had been blocked. The appropriateness of the location of the altered sensation, however, is not as easily deter-

406

A.J. GABOR

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Fig. 2. EEG corresponding to Fig. I. A and demonstrating rhythnaic slow waves (underlined} in right ccntro-parietai area with superimposed muscle artifact. Calibration for this and subsequent figures: I sec. 50 I~V.

Fig. 3. EEG corresponding to Fig, I, B and demonstrating rapid rhythmic 12see spikes in right central area with some later involvenlent o~" homologous areas on the left. For calibration see Fig. 2.

mined directly and only indirect evidenc- must be considered. Such evidence depends ~.avily upon the manner in which the seizures may be provoked as well as the pre-ictal and pos~ctal clinical manifestations. In this regard, since only movements of the left hand provoked the seizures, and the earliest maniii:station of the seizure (initially distal sensory then motor) involved exclusively the left hand and the postictal weakness was most marked in the distal left hand, one may provisionally speculate that if peripheral sensory mechanisms did provide the "'trigger", such impulses would originate in the left arm, probably in the more distal areas. If such speculation is accepted, then the ability to provoke seizures after brachial blockade and the inability to provoke seizures by passive movements indicate the non-essential nature of peripheral sensory mechanisms in some seizures induced by movement. It is therefore likely that central mechanisms may be capable of either replacing peripheral sensory triggers or may be solely responsible for some seizures in-

duced by movement. The location of these central mechanisms may be spinal, brain stem or cortical and this study provides no information in this regard. The alternative explanation is that the sensory information crucial to epileptogenesis did not reside in the left arm: however, the inability to produce seizures by passive or ~ctive movement of other parts of the body makes this notion unlikely. In the context of movement induced seizures this case is unique. Only exceptional reports (Burger et al. 1972) have described seizures provoked by sustained, repeated muscular activity and in such cases the activity has been described as generalized muscular exertion rather than being restricted to a single limb. The case described by Falconer et aL {1963) is similar with respect tc '~e restricted nature of the movement, but differed in that the movement provoking the seizures had to be sudden rather than sustained and repetitive. Finally, the seizure was manifest by a localized tonic/clonic sequence with its

FOCAL SEIZURES INDUCED BY MOVEMENT

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Fig, 5. Beginning of seizure after brachial block. Compare early rhythmic slow waves in right cemral region with those in Fig. 2. Fur calibration see Fig. 2.

appropriate electrographic correlate rather than being strictly tonic as generally reported (Kertesz 1967) with generally poor electrographic correiation (see Falconer et aL 1963: Perez-Borja et al. 1967 as exceptions). Finally, cerebral localization of the seizure may be considered. While the early sensory and motor manifestations during the course of the seizure are typical of an origin in or near the supplementary motor area (Ajmone Marsan and Ralston 1957), other features, however, are more difficult to reconcile with such a location. The electrographic changes occurring in the interictal state and concomitantly with the earliest subjective manifestation of the seizure were maximal in the centro-parietal region consistent with an origin on the cortical convexity. The lower extremity was never involved in the seizure and a generalized seizure never occurred, as one might have expected from a lesion within the sagittal fissure. The distal arm was primarily involved both in the pre-ictai and postictal state. Based upon these considerations an epileptogenic focus on the cerebral convexity near the Rolandic

fissure seems most likely, with possible secondary involvement of the supplementary motor area. If such a localization may be considered correct in the absence of a demonstrable structural lesion, it must be presumed the other cortical areas besides the supplementary motor area may be suspected as the origin of the seizures in cases with seizures induced by movement. SUMMARY

A 49-year-old right-handed female developed repeated tonic-clonic seizures involving only the left arm and face during an episode ofnon-ketotic hyperglycemia. These seizures were documented cinematographically and electrographically. They occur, ed spontaneously but could also be provoked by repeated movements of the left hand. A complete left brachial anesthetic block was produced, but a seizure could still be provoked by the patient attempting to move the left hand. The seizure was thought to originate from the right cerebral convexity near the Rolandic fissure and was found not to be dependent on peripheral sensory impulses.

408 RESUME CRISES FOCALISEES INDUITES PAR LE MOUVEMENT SANS MECANISME DE.Iced-back SENSITIF

Une femme droiti~re ag6e de 49 ans a pr6sent6 des crises tonico-cloniques r~p6t6es n'impliquant que le bras gauche et la lace au cours d'un 6pisode d'hyperglyc6mie non-k6totique. Ces crises ont 6t6 cin6matographi6es et enregistr6es fi I'EEG. Elles surviennent spontan6ment mais peuvent 6galement 6tre provoqu6es par des mouvements r6p6t6s de la main gauche. Un blocage anesth6sique bracchial gauche complet a ~t6 r6alis6, mais une crise a pu encore ~tre provoqu6e par une tentative par la malade de mouvoir la main gauche. L'auteur pense que la crise prend son origine h partir de la convexit6 c~r6brale droite proche de la scissure de Rolando et qu'elle ne d6pend pas d'aff~rences sensitives p6riph6riques.

The assist, nee ot'John H. Eisele. M.D. of the Department of Anesthesiology and the technical assistance of Betty Rasor are gratefully acknowledged.

A.J. GABOR REFERENCES

AJMONE MARSAN. C. and RALSTON, B. L. Tile epileptic sci:m'£,. Its .lUnctiomd morphology and diagnostic siffni/i¢'a!we. Thomas, Springfield. Ill., 1957. ALA.~'"UANINE, T. et GASTAUT. H. La syncin~sie-sursaut el iepilepsie-sursaut fi d~clenchement sensoriei ou sensitif inopin6. Les faits anatomo-cliniques ( 15 observations). Rer. neurol., 1955, 93:29-41. ALAJOUANINE. T. et GASTAUT, a . La syncin~sie-sursaut et I'~pilepsie-sursaut ",i -i,h=lenchement sensoriel ou sensitif inopin~. Consid6rafions sur les ~pilepsies dits r~flexes. In Bases physioloqiques et aspects cliniques de I'epilepsie. Masson et Cie, Paris, 1958: 199-231. BURGI-R. L. J., LOPEZ. R. i. and ELLIOT, F. A. Tonic seizures induced by movement. Neuroloqy ¢Minneap. ). 1972, 22: 656- 659. FALCONER. M. A., DRIVER. M. V. and SERAFE'n.~mm, E. A. Seizures induced by movement: Report of a case relieved by operation. J. Neurol. Neurosurq. Psychiat., 1963, 26: 3O0-3O7. GASTAUr. H. and TASSINARI,C. A. Triggering mechanisms in epilepsy. The electro-clinical point of view, Epih,psh+ (,4rest.~, 1966, 7: 85+ 138. KF,RrEsz. A. Paroxysmal kinesigenic choreoathetosis, Neum~h~.q.v ( Mhmeap. ), 1967, 17: 680-690. LISlIMan, W. A.. SYMONDS. C. P., WIIlTTY. C. W. M. and WtLt+ISON, R. G. Seizures induced ~,.y movement. Brain, 1962, ,'¢5:93 108. PrRI,.'z-BOaJA. C., TASSINARL A. C. and SWANSON, A. G. Paroxysmal choreoathetosis and seizures induced by movement, EpihTsh+ (,4ms: ,, 1967, ,"¢: 260 270. Srv vENs, H. Paroxysmal choreo-athetosis. Arch. NeuroL (Chh.,). 1966,14: 415-420. WHIrT'c. C. W. M., LlSHMAN, W. A. and FI-tZGInm~g, J, P, Seizutg~ induced by movement: A form of reflex epilepsy, Lam'et, 1964, I: 1403 141)5,