Medial Temporal Lobe Epilepsy with Severe Pain Sensation

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1 Copyright 2009 American Scientific Publishers All rights reserved Printed in the United States of America American Journal of Neuroprotection and Neuroregeneration Vol. 1, 1 5, 2009 Medial Temporal Lobe Epilepsy with Severe Pain Sensation P. Kolev 1, N. Mihnev 1, D. Maslarov 2, and M. Markov 1 1 University Hospital of Neurology and Psychiatry St. Naum, Medical University, Sofia, Bulgaria 2 1st MHAT-Department of Neurology, Sofia, Bulgaria Ictal pain is one of several types of somatic sensations felt during partial seizures. As a sole symptom, ictal pain is extremely rare and it is uncommon for temporal lobe epilepsy. In a case report we present a patient with temporal lobe seizures with the basic symptoms of severe left leg pain and muscles cramps. A 29-year-old man was admitted to the hospital because of probable epilepsy. After sport trauma (left leg injury) 10 years ago, he had seizures of severe pain 1 4 times daily, muscle cramps spread throughout his left leg, often saw stars after physical overfatigue and when he was feeling cold. Seizures lasted one to two minutes. He had never lost consciousness. Seven years ago he was misdiagnosed as having orthopaedic disorder. In the last two years these complaints were rarely preceded by lower lip trembling. He had no family history of epilepsy. The treatment with carbamazepine (CBZ) monotherapy, valproate (VPA) monotherapy and combination CBZ + VPA did not show any seizure reduction. Routine interictal EEG recordings showed normal background activity. Video-EEG revealed ictal onset consisted of rhythmic 7.5 Hz activity of theta waves over the left midtemporal region, which spread to the right midtemporal region, followed by diffuse spike-waves with 7 Hz, but predominated on the left midtemporal. It was the clinical start of the seizure. During the seizure the patient had a tortured face and screamed, because of the pain. There was no consciousness impairment and postictal confusion. The seizure lasted one minute. Fluid-attenuated inversion recovery (FLAIR) magnetic resonance and interictal SPECT demonstrate left hippocampus changes. We started treatment with CBZ 600 mg/daily and levetiracetam (LEV) 2500 mg/daily, seizures reduced to only once a month. This case shows that seizure semiology of temporal lobe epilepsies could be quite different and often misdiagnosed as other disorders. Keywords: Ictal Pain, Temporal Lobe Epilepsy, Hippocampal Sclerosis, Treatment of Epilepsy. 1. BACKGROUND Ictal pain is one of several types of somatic sensations felt during partial seizures, and often accompanies other sensations like tingling paresthesias, thermal sensations, and sexual sensations. Central pain of epileptic etiology can occur very rarely. The frequency of painful sensations in epileptic seizures varies between 0.3 and 2.8%. 1 2 Epileptic pain can be experienced anywhere in the body and, based on the principal location, is divided into three categories: lateralized peripheral, cephalic, and abdominal. Most patients report unilateral sensations, but bilateral pain also has been described. 3 Epileptic pain is commonly caused by epileptic discharges in the parietal lobe, but temporal lobe seizure origin also has been postulated. Although epileptic pain is usually associated with other seizure symptoms, it can sometimes be the only Author to whom correspondence should be addressed. manifestation of epilepsy. 4 In such cases, seizures are often misdiagnosed, and patients go through unnecessary diagnostic procedures until the correct diagnosis is made. 5 Among somatosensory seizures, Mauguiere and Courjon 4 found pain in 23.6% of the seizures. In several recent reports of parietal lobe epilepsy, 6 8 pain is described in only one patient each. Seizure onset for both of the ictal pain groups is most common in the first two decades. 5 9 Painful unilateral sensations of the extremities are most commonly experienced as burning, dull, throbbing, crampor electric-like. Ictal pain may be felt anywhere in the body, but the most common locations are a part or the whole of one side, the head (cephalic), and the abdomen. 10 Authors reported the duration of the unilateral ictal pain to be between few seconds 5 and several minutes. 7 Subsequent studies in humans using functional imaging techniques (PET, SPECT, and functional MRI) showed several structures to be involved in pain perception. These Am. J. Neuroprotec. Neuroregen. 2009, Vol. 1, No /2009/1/001/005 doi: /ajnn

2 Medial Temporal Lobe Epilepsy with Severe Pain Sensation Kolev et al. structures are contralateral anterior cingulate gyrus, 11 contralateral supplementary motor area (within area 6), contralateral anterior (probably disgranular) and bilateral posterior (probably granular) insular cortex, 12 both contralateral and ipsilateral thalamus, ipsilateral putamen, cerebellar vermis, and bilateral periaqueductal grey/dorsal midbrain. 13 Unilateral paraesthetic seizures are considered the clinical expression of involvement of contralateral somatosensory primary area. 4 Bilateral paraesthetic seizures, with or without acoustic sensation, are due to involvement of secondary or supplementary sensory areas CASE REPORT We present a patient with temporal lobe seizures with basic symptoms of severe left leg pain and muscles cramps. A 29-year-old man was admitted to the hospital because of probable epilepsy. After sport trauma (left leg injury) 10 years ago, he had seizures of severe pain 1 4 times daily, muscle cramps spread throughout his left leg, often saw stars after physical overfatigue and when he was feeling cold. Seizures lasted one to two minutes. He had never lost consciousness. Seven years ago he was misdiagnosed as having orthopedic disorder and was unsuccessfully treated with analgesics and anti-inflammatory medications. In the last two years these complaints were rarely preceded by lower lip trembling. He had no family history of epilepsy. The treatment with carbamazepine (CBZ) monotherapy, valproate (VPA) monotherapy, and a combination of CBZ and VPA did not show any seizure reduction. Routine interictal EEG recordings showed normal background activity. Video-EEG revealed ictal onset consisted of rhythmic 7.5 Hz activity of theta waves over the left midtemporal region- F7 T3 and T3 T5 (Fig. 1), which spread to the right midtemporal region F8 T4 and T4 T6 (Fig. 2), followed by diffuse spike-waves with 7 Hz (Fig. 3), but predominated on the left midtemporal. It was the clinical start of the seizure. During the seizure the patient had a tortured face and screamed because of the pain. There was no consciousness impairment and postictal confusion. The seizure lasted 1 minute. Fluid-attenuated inversion recovery (FLAIR) magnetic resonance demonstrated unilateral gliosis of the left hippocampus (Fig. 4). Interictal SPECT with TC-99m- HMPAO (Fig. 5) demonstrates decreased uptake on the left hippocampus compared with the rest of the brain (cold focus). The areas that showed decreased perfusion correlated with EEG and MRI findings. Initially, we started treatment with CBZ (600 mg/daily) and LEV (2000 mg/daily). One month after the recommended dosage was reached, seizures significantly decreased to only once a month. Because of these improvements, but still incomplete treatment effect, we increased levetiracetam (LEV) to 2500 mg/daily and no painful seizures occurred. 3. DISCUSSION In the reported cases, somatosensory seizures often appeared as aura and they are described as tingling, numbness, electric shock sensation, pain, sense of movement, or desire to move. 15 In the case with our patient, the ictal pain was the sole symptom of temporal lobe epilepsy for a long time. The most frequent somatic topography in unilateral seizures are the hands and fingers. Arm, Fig. 1. Video-EEG revealed ictal onset consisted of rhythmic 7.5 Hz activity of theta waves over the left midtemporal region- F7 T3 and T3 T5. 2 Am. J. Neuroprotec. Neuroregen. 1, 1 5, 2009

3 Kolev et al. Medial Temporal Lobe Epilepsy with Severe Pain Sensation Fig. 2. Ictal changes spread to the right midtemporal region F8 T4 and T4 T6. foot, leg, face, head, and trunk may also be the loci of initial symptoms. 16 Tingling is the most frequent sensation due to focal epilepsy, constituting about half of the cases. 17 Patients with somatosensory symptoms usually present focal motor seizures, with clonic, tonic, or postural movements. 17 Somatosensory seisures have been reported in parietal epilepsy and they can be present in other focal epilepsies. Frontal and temporal lobe epilepsies may also be presented with somatosensory seizures. 18 Although mesial temporal lobe epilepsy is the most common form of temporal epilepsy in adults, in some patients it remains unrevealed. Our literature review showed that the first seizure usually occurs in late childhood or early adolescence. In the case with our patient seizures began later. Somatosensory seizures of extremities most often occur contralateral to the side of ictal onset. In our patient ictal pain is ipsilateral on seizure focus. Seizures typically last about one minute or less. Interictal electroencephalogram findings in patients with mesial temporal lobe epilepsy typically include unilateral or bilaterally-independent mesial temporal spikes. In our patient interictal EEG recordings showed nothing abnormal. Ictal EEG recordings usually reveal ictal onset consisting of rhythmic 5 to 7 Hz activity in one midinferomesial temporal region, but there may be variations in this pattern. High-resolution MRI often demonstrates unilateral or bilateral hippocampal atrophy associated with hypertintense signal in one or both hippocampi sometimes extending to the amygdala or other medial temporal structures. 22 Fluid-attenuated inversion recovery (FLAIR) Fig. 3. Clinical start of the seizure: diffuse spike-waves with 7 Hz, predominantly on the left midtemporal. Am. J. Neuroprotec. Neuroregen. 1, 1 5,

4 Medial Temporal Lobe Epilepsy with Severe Pain Sensation Kolev et al. Fig. 4. Fluid-attenuated inversion recovery (FLAIR) magnetic resonance demonstrated unilateral gliosis of the left hippocampus. magnetic resonance images of the left hippocampus in our patient showed increased signal intensity. Mesial temporal sclerosis identified by MRI, had been associated with poor control of seizures by antiepileptic medication. Our patient had good seizure control with CBZ and LEV. However, the findings of MRI abnormalities in patients with good outcome or seizure remission, indicates that mesial temporal sclerosis is found not only in patients with medically refractory mesial temporal lobe epilepsy Furthermore, there is upcoming MRI evidence of mesial temporal sclerosis acquired in adulthood that is not necessarily associated with a poor seizure control. 26 Fig. 5. Interictal SPECT with TC-99m-HMPAO demonstrates decreased uptake on the left hippocampus compared with the rest of the brain (cold focus). The areas that showed decreased perfusion correlated with EEG and MRI findings. 4. CONCLUSION Painful unilateral sensations of the extremities as ictal simptoms of temporal lobe epilepsy are very rare. Epileptic pain is usually associated with other seizure symptoms, but sometimes it could be the sole manifestation of epilepsy. Therefore, seizures are often misdiagnosed, and patients go through unnecessary diagnostic procedures before the correct diagnosis is made. References and Notes 1. J. Scholz, P. Vieregge, and A. Moser, Pain Mar. 80, 445 (1999). 2. G. B. Young and W. T. Blume, Brain 106, 537 (1983). 3. K. Otani, K. Imai, Y. Futagi, and K. Yanagihara, Dev. Med. Child Neurol. 37, 933 (1995). 4. F. Mauguiere and J. Courjon, Brain 101, 3072 (1978). 5. M. E. Lancman, J. J. Ascoape, K. T. Penry, and T. Brotherton, Pediatr. Neurol. 9, 404 (1993). 6. P. D. Williamson, P. A. Boon, V. M. Thadani et al., Ann. Nemo. 31, 193 (1992). 7. G. D. Cascino, J. F. Hulihan, F. W. Sharbrough, and P. K. Kelly, Epilepsia 34, 522 (1993). 8. S. S. Ho, S. F. Berkovic, M. R. Newton, M. C. Austin, W. J. McKay, and P. F. Bladin, Neurology 44, 2277 (1994). 9. J. S. de Lope, O. A. Fernandez, J. A. Mendoza, J. B. Coronel, F. Barinagarrementeria, and J. P. Manauta, Rev. Gastroenterol Mex. 59, 297 (1994). 10. R. R. Babb and P. B. Eckman, JAMA 222, 65 (1972). 11. R. C. Coghill, J. D. Talbot, A. C. Evans et al., J. Neurosci. 14, 4095 (1994). 12. K. Ostrowsky, M. Magnin, P. Ryvlin, J. Isnard, M. Guenot, and F. Mauguière, Cereb. Cortex 12, 376 (2002). 13. B. A. Vogt, S. Derbyshire, and A. K. Jones, Eur. J. Neurosci. 8, (1996). 14. G. Wunderlich, M. F. Schüler, A. Ebner, H. Holthausen, I. Tuxhorn, O. W. Witte, and R. J. Seitz, Epilepsy Res. 38, 139 (2000). 15. W. T. Blume, H. O. Lüders, E. Mizrahi, C. Tassinari, W. E. Boas, and J. Engel, Jr., Epilepsia 42, 1212 (2001). 16. D. R. Nair, I. Najm, J. Bulacio, and H. Lüders, Neurology 57, 700 (2001). 4 Am. J. Neuroprotec. Neuroregen. 1, 1 5, 2009

5 Kolev et al. 17. I. E. B. Tuxhorn, Seizure 14, 262 (2005). 18. D. W. Kim, S. K. Lee, C. H. Yun, K. K. Kim, D. S. Lee, C. K. Chung, and K. H. Chang, Epilepsia 45, 641 (2004). 19. F. Cendes, P. Kahane, M. J. Brodie, and F. Andermann, The mesiotemporal lobe epilepsy syndrome, Epileptic Syndromes in Infancy, Childhood and Adolescence, 3rd edn., edited by J. Roger, M. Bureau, C. Dravet, P. Genton, C. A. Tassinari, and P. Wolf, John Libbey & Co Ltd, Eastleigh, UK (2002). 20. E. Kobayashi, I. Lopes-Cendes, C. A. Guerreiro, S. C. Sousa, M. M. Guerreiro, and F. Cendes, Neurology 56, 166 (2001). Medial Temporal Lobe Epilepsy with Severe Pain Sensation 21. J. C. Erickson, L. E. Clapp, G. Ford, and B. Jabbari, Epilepsia 47, 202 (2006). 22. N. Foldvary, B. Nashold, and E. Mascha, Neurology 54, 630 (2000). 23. E. Cendes, Curr. Opin. Neurol. 18, 173 (2005). 24. W. J. Kim, S. C. Park, S. J. Lee, J. H. Lee, J. Y. Kim, B. I. Lee et al., Epilepsia 40, 290 (1999). 25. E. Kobayashi, L. M. Li, I. Lopes-Cendes, and E. Cendes, Arch. Neurol. 59, 1891 (2002). 26. E. Cendes, Curr. Opin. Neurol. 17, 161 (2004). Received: 15 September Accepted: 15 March Am. J. Neuroprotec. Neuroregen. 1, 1 5,