7147ournal of Neurology, Neurosurgery, and Psychiatry 1992;55:714-716 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.55.8.714 on 1 August 1992. Downloaded from SHORT REPORT Prolonged sensory or visceral symptoms: an under-diagnosed form of non-convulsive focal (simple partial) status epilepticus
M Manford, S D Shorvon
Abstract slow waves, negative in the right frontocentral Four patients had prolonged, sensory, leads. Postictally the continuation of the but- simple partial seizures (SPS), lasting up terfly sensation was characterised by a period to several days, without associated behav- of frontocentral, electrographic status epilepti- ioural impairment. In three patients, the cus (figure). During this episode there was no SPS often occurred as a prolonged objective behavioural abnormality. "aura" before a more overt seizure. Intracranial ictal EEG revealed a build up of Descriptions included: "butterflies", ris- spike and wave at the deepest medial frontal ing epigastric sensation; "a thought in the electrode, which spread to the rest of the stomach", and an olfactory sensation. frontal lobe, to the temporal neocortex and to Seizure localisation was frontal in one the hippocampus. There were frequent, wide- case, temporal in two cases and uncertain spread, interictal spikes that were not clearly in one case. These sensations may repre- correlated with the butterfly sensation. He did sent an under-reported form of continu- not, however, experience such a prolonged ous, focal seizure activity, which arises feeling as during scalp recording. from various cerebral regions. He had right frontal lobectomy with intra- operative electrocorticography. The resected (7 Neurol Neurosurg Psychiatry 1992;55:7 14-716) tissue showed histological evidence of a neu- ronal maturation defect. One year later, he had suffered a single cluster of seizures, and no The epileptic aura is generally considered to be further prolonged auras. a short-lived phenomenon, representing the early stages of build up of abnormal, synchro- Case 2: "Thought in the stomach". nous electrical activity. This usually develops Case 2, a twenty five year old, right handed further into a more obvious seizure or else halts male motor mechanic had a normal neonatal abruptly, with reversion to the interictal state. and early childhood history. At five years of age We present four cases of prolonged, sensory he suffered a GTCS without obvious precipi- SPS, without invariable ictal progression, pre- tant. He was treated with phenobarbitone, and senting to us over the course of one year. had no further attacks until the age of eleven http://jnnp.bmj.com/ years, when he started to experience CPS. These started with an aura of a "thought in his stomach", lasting up to one minute. A brief Case reports speech arrest with blankness, was followed by Case 1: "Butterfly sensation" hand rubbing automatisms, dysphasia and This was a twenty three year old, right handed amnesia, lasting a few minutes. He was seizure
male motor mechanic. Complex partial seiz- free from 16-20 years of age, but seizures then on September 25, 2021 by guest. Protected copyright. ures (CPS) first occurred when he was six recurred in the same pattern. When he was 24 years old, in association with fever and a head years old, his medication was changed and his injury but they did not become regular until he overt seizures ceased. However, at roughly was 10. They were frequently preceded, for up monthly intervals (the same frequency as his to two days, by a butterfly sensation, often felt seizures one year previously) he experiences in the abdomen but not specifically localised, the same sensation of a "thought in his Institute of Neurology, which sometimes persisted after the seizure. stomach". This fluctuates over several days, National Hospitals for Attacks were recorded with video-EEG-tele- not disappearing and not reaching the severity Neurology and metry, before and after stereotactic EEG elec- he associates with an impending attack. Dur- Neurosurgery and The National Society for trode insertion. They started with dizziness ing this time he remains fully alert and able to Epilepsy Research and pallor, followed by a single loud scream, carry out his normal work and duties. Group, Chalfont abduction of his legs, bilateral upper limb Interictal EEG consistently showed mild M Manford com- over S D Shorvon posturing, then rocking movements and sharp and slow wave abnormalities the plex motor activity, for example, bicycling right anterior temporal region. CT and MRI Correspondence to: Dr Manford, Institute of movements. After their recurrence, CPS were normal. We have not obtained an EEG Neurology, Queen Square, occurred in clusters up to one an aura. London WClN 3BG, UK lasting week, during with a cluster often followed a Received 23 August 1991 by generalised and in revised form tonic-clonic seizure (GTCS). Case 3: Epigastric sensation 11 November 1991. Ictal EEG showed a 20 This was a 36 year old right handed, male Accepted 20 November scalp recording 1991 second build up of high amplitude sharp and housing officer with a normal perinatal and Prolonged sensory or visceral symptoms: an under-diagnosed form of non-convulsive focal (simple partial) status epilepticus 715 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.55.8.714 on 1 August 1992. Downloaded from EEG (P4/A2) __4 o o -VW EEG (Cz/Oz) Left eye
Right eye VA%4\A. Chin EMG
Left ant tib. Right ant tib. I-P I 4 6 Left nostril Right nostril ECG 1 sec Figure I Case 1: Standard 10-20 scalp EEG during a prolonged epigastric sensation, showing rhythmical slow activity, predominantly in the right frontocentral leads.
early childhood history. Seizures started at the carbamazepine made no impact on his attacks, age of 18 and have been present once a week and subsequently phenytoin was only tran- for the past 13 years. On the day he is due to siently effective. have a seizure, he awakes with an epigastric sensation, which persists for hours and a few seconds before an attack, it rapidly rises to his Discussion chest. He shouts "Oh God" and is unaware for An aura represents a focal epileptic discharge, several seconds. There follows up to one manifesting as a sensory SPS, which may minute of manual automatisms. Recovery progress to a more overt seizure. The first three occurs within two minutes, with no dysphasia. cases clearly satisfy the International League Sometimes the epigastric sensation persists Against Epilepsy criteria for an aura;' a ster- after the seizure when he knows he will have a eotyped sensation, without impairment of further seizure that day. consciousness, temporally closely related to CT scan was normal but MRI, with planes seizures. The fourth is also epileptic in view of orientated parallel to the long hippocampal clinical features, EEG and MRI findings and axis, showed substantial loss of right response to anticonvulsants. They are, how- hippocampal substance. Interictal EEG on a ever, unusual in that the phenomena may normal day showed diffuse delta and sharper persist for a considerable period without devel- components anteriorly with a left-sided pre- opment of an overt seizure. This can be http://jnnp.bmj.com/ ponderance. Video telemetry was performed: classified as "simple partial status epilepticus", there was no EEG abnormality during an aura non-convulsive as distinct from epilepsia par- or a seizure, but postictally there was diffuse tialis continua (EPC) or other motor seizure right sided slow activity. phenomena. For case 1, the sensation was clearly asso- Case 4: Olfactory sensation ciated with electrical seizure activity on surface
A right handed 55 year old male electronics but not on intracranial EEG. Up to half of on September 25, 2021 by guest. Protected copyright. engineer had experienced occasional microp- auras may be missed on intracranial recording, sia, with the illusion of progressive diminution probably because of the small volume of tissue of the size of objects since the age of four years. sampled.2 'The diagnosis of an aura is clinical; This lasted for two minutes, with no alteration not affected by the absence of localising EEG of consciousness and was associated with a abnormality, either ictal (cases 3, 4) or inter- pleasurable sensation. Since 1984 he has suf- ictal (case 2). fered frequent, strong, olfactory hallucinations Evidence for a frontal focus is strong in case of a variable nature, but usually a chemical 1, and cases 3 and 4 strongly suggest temporal odour, resembling paint or hot oil. These lobe epilepsy. These results present evidence would persist for several days with numerous that prolonged, sensory SPS is not a property exacerbations lasting up to 30 minutes. There of a single cerebral region. have not been any more overt seizures and his Psychomotor complex partial and focal work and concentration remain unimpaired. motor status epilepticus are well accepted Interictal EEG consistently showed a left- entities, but despite series amounting to thou- sided spike focus between the anterior and sands of cases with careful analysis of seizure midtemporal regions, which did not change in semiology," ' we have found few reports of association with the hallucinations. CT scan sensory status epilepticus. Hughlings-Jack- was normal but volumetric MRI showed son"' described a case in which: "sometimes reduction in volume of the anterior portion of when the attacks are about, she has a sensation the left hippocampus. Initial treatment with of smell or of taste at the back ofher throat, but 716 Manford, Shorvon
not in connection with her seizures". Penfield therefore be a relatively benign form of pro- J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.55.8.714 on 1 August 1992. Downloaded from and Jasper" described a patient with a pre- longed seizure activity. If, as has been sug- vious parietal injury who suffered continual gested, continuous, focal discharges are tingling of the left foot for a week and recently, harmful to neurons in the long term,"3 then Sowa and Pituck'2 reported a case with weeks early identification and treatment of this group of continuous visual hallucination, accom- may prevent the development of a more panied by EEG abnormality and with no intractable seizure disorder, and associated functional impairment. Our four patients were neuropsychological deterioration. taken from our current clinical practice and we have seen others with a past history of a similar 1 Proposal for revised clinical and electroencephalographic classification of epileptic seizures. From the Commission phenomenon, suggesting that this pattern of on Classification and Terminology of the International League Against Epilepsy. Epilepsia 1981 ;22:489-50 1. epilepsy may be more frequent than previously 2 Sperling MR, Lieb JP, Engel J Jr, Crandall PH. Prognostic recognised. These patients with a prolonged, significance of independent auras in temporal lobe seizures. Epilepsia 1989;30:322-31. continuous SPS are distinct from others who 3 Sperling MR, O'Connor MJ. Auras and subclinical seizures: experience frequent, brief auras before an Characteristics and prognostic significance. Ann Neurol 1990;28:320-8. overt seizure. 4 Janati A, Nowack WJ, Dorsey S, Chesser MZ. Correlative These symptoms represent continuous, study of interictal encephalogram and aura in complex partial seizures. Epilepsia 1990;31:41-6. highly localised seizure activity, manifesting as 5 Gowers WR. Epilepsy and other chronic convulsive diseases: sensory or visceral symptoms, without spread their causes, symptoms and treatment. London: Churchill 1901. to areas that would result in behavioural 6 LennoxWG, Cobb S. Aura in epilepsy; a statistical review of impairment. The subtlety of these symptoms, 1,359 cases. Arch Neurol Psychiat 1933;30:374-87. 7 Gupta AK, Jeavons PM, Hughes RC, Covanis A. Aura in that they last longer than is usual for epileptic temporal lobe epilepsy: clinical and electroence- phenomena and that they are rarely accom- phalographic correlation. J Neurol Neurosurg Psychiat 1983;46: 1079-83. panied by EEG change, probably explain why 8 Taylor DC, Lochery M. Temporal lobe epilepsy: origin and they are less frequently recognised than other significance of simple and complex auras. J Neurol Neurosurg Psychiat 1987;50:673-81. forms of focal status epilepticus, for example, 9 Kanemoto K, Janz D. The temporal sequence of aura- EPC or psychomotor. This may mean that a sensations in patients with complex focal seizures with particular attention to ictal aphasia. Y Neurol Neurosurg group ofpatients has undetected, mildly symp- Psychiat 1989;52:2-56. tomatic, prolonged seizure activity; case 4 had 10 Selected writings ofJohn Hughlings-Jackson vol 1. ATaylor, a ed. London: Staples Press, 1958:385-405. symptoms from early childhood, but diag- 11 Penfield W, Jasper H. Epilepsy and the functional anatomy of nosis was only made in middle age. the human brain. Boston: Little and Brown, 1954:395. had severe 12 Sowa MV, Pituck S. Prolonged spontaneous complex visual Apart from patient 1, who also hallucinations and illusions as ictal phenomena. Epilepsia complex partial seizures, all these patients were 1989;30:524-6. 13 Rodin EA, Schmaltz S, Twitty G. Intellectual functions of in employment and continuing to function patients with childhood onset epilepsy. Dev Med Child normally in their social setting. This may Neurol 1986;28:25-33. http://jnnp.bmj.com/ on September 25, 2021 by guest. Protected copyright. Matters Arising 1 223
patients with chronic lumbosacral disc dis- mal sensitivity. Funct Neurol 1991:6:125-31. gress Secretariat ISRS 1993, Stockholm eases as described in our study. We think 3 Galfe G, Lautenbacher S, H61zl R, Strian F. Diagnosis of small fibre neuropathy: com- Convention Bureau, PO Box 6911, S-102 38 there are important differences in pathophy- puter-assisted methods of combined pain and Stockholm, Sweden. siology. So far the testing of neuropathic thermal sensitivity determination. Hospimedi- conditions with heat pain stimulation has ca 1990:8:38-48. in 4 Jensen TS, Bach FW, Kastrup J, Dejgaard A, rarely resulted strong evidence for hyper- Brennum J. Vibratory and thermal thresholds algesic or hyperpathic changes. In a very in diabetics with and without clinical neurop- recent publication,' however, Wall gave a athy. Acta Neuml Scand 1991:84:326-33. great number of examples of hyperalgesic 5 Wall PD Neuropathic pain and injured nerve: Ciba-Geigy * ILAE * IBE - Epileptology central mechanisms. Br Med Bull 1991: Prize changes produced by different kinds of neu- 47:631-43. ropathies and he also pointed to the fact that a non-selective blockade of peripheral affer- Ciba-Geigy has agreed with the International ent impulses may lead to a "partial disinhibi- League Against Epilepsy (ILAE) and the tion" and, in consequence, to hyperalgesia. NOTICES International Bureau for Epilepsy (IBE) to This is what seemed to have happened in our establish a new prize of SFr. 20.000. to be patients with chronic lumbosacral disc dis- awarded in recognition of outstanding ease. That such an event might produce achievement in the field of epilepsy. The prize effects at the contralateral side appears not Tropical Neurology Symposium, Lon- is designed to encourage applied human too speculative when the results of con- don, 25 March 1993 research in epilepsy. By cooperating with tralateral TENS-effects cited in our paper are both ILAE and IBE, Ciba-Geigy emphasises considered. Taken together, we still believe The first Tropical Neurology symposium to that application for the prize is open to that the conclusions drawn from the pilot take place in the United Kingdom will be candidates from all fields of applied study described are justified. held at Manson House, 26 Portland Place, research. Finally, we want to answer the questions London. It will be co-sponsored by the Anyone outside the pharmaceutical indus- raised by Bowsher. Two patients were affec- Tropical Neurology Research Group of the try who considers that he or she has made a ted at the L5 root affection and 7 at the S1 World Federation of Neurology jointly with significant scientific contribution in the field root. We measured the thresholds at the the Royal Society of Tropical Medicine and of epilepsy may compete for the prize. medial (L5) and lateral (S1) side of the Hygiene. It will be chaired by Lord Walton, Deadline for submission is 15 January dorsum pedis, where the peripheral derma- Professor D Warrell, Professor N Wadia and 1993. tomes are to be found, and verified the Dr CM Poser. Entries for the prize will be judged by an location of the dermatomes in the preceding Further details from: The Administrator, Adjudicatory Panel consisting of the Presi- neurogical examination. As Bowsher expec- Royal Society of Tropical Medicine and dents and one further delegate each of ILAE ted, the dermatones do normally not differ in Hygiene, Manson House, 26 Portland Place, and IBE, and an independent chairman. warmth and pain sensitivity. London WIN 4EY. Telephone: 071-580 The prize will be awarded at the opening S LAUTENBACHER 2127; Fax: 071-436 1389. ceremony of the International Epilepsy Con- Max Planck Institut ffir Psychiatrie, gresses (for the first time in Oslo on 3 July Kraepelinstr 10, D-8000 1993, and subsequently in Sydney in Munich, Germany First Congress ofthe International Ster- 1995). eotactic Radiosurgery Society, Stock- For further details and a set of the rules 1 Strian F, Lautenbacher S, Galfe G, Holzl R. holm, Sweden and application forms, those interested Diurnal variations in pain perception and thermal sensitivity. Pain 1989;36:125-31. should write to Mrs. G Haldemann, Ciba- 2 Lautenbacher S, Strian F. Similarities in age This congress will be held from 16-19 June Geigy Limited, CH-4002 Basel, Switzer- differences in heat pain perception and ther- 1993. Further information from The Con- land.
Correction toms: an under-diagnosed form of non- nal), the figure was incorrect. The figure In the article by M Manford and SD Shor- convulsive focal (simple partial) status should have been as follows: von, Prolonged sensory or visceral symp- epilepticus (August 1992 issue of the jour-
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F3-C3 \ -tW, -tv-tv- -Q \>1 Figure Case 1: Standard 10-20 scalp EEG during a prolonged epigastric sensation, showing rhythmical slow activity, predominantly in the right frontocentral leads.