A case of MELAS presenting complex partial status epilepticus]

We reported a 37-year-old man who presented complex partial status epilepticus as the initial symptom of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS). He showed fluctuating consciousness disturbance, left homonymous hemianopsia, and paroxysmal conjugated eye deviation to the left. The lactic acid level was elevated in blood and CSF, and ragged-red fibers were observed in the biopsied muscle. MRI demonstrated T2-prolonged lesions in the right occipito-parieto-temporal lobes. Since a mutation of mitochondrial DNA (A3243G) was identified, he was diagnosed as having MELAS. On an ictal record, high amplitude, rhythmic sharp waves were observed at right parieto-temporo-occipital region. High amplitude slow waves were also observed on the right hemisphere, especially in the right frontal lobe. These ictal discharges gradually decreased at their amplitude and in frequency, and then ictal EEG turned to the interictal EEG. During an ictal period, conjugated eye deviation to the left side and consciousness loss were observed. These seizures were observed once every several minutes. During the interictal period, sharp waves and sharp-wave complexes were observed frequently at right parietal and posterior temporal lobes. The venous injection of diazepam (10 mg) normalized EEG quickly. When consciousness loss, especially fluctuating, was observed in the patients of MELAS, complex partial status epilepticus should be considered.     

Ictal MEG in two children with partial seizures

We report on the successful identification of epileptic foci in two children with partial epilepsy using ictal magnetoencephalography (MEG). Case 1 is a 12-year-old male suffering with simple partial seizures with leftwards nystagmus. Ictal SPECT revealed a hyperperfusion area in the right lateral occipital area, and MRI revealed cortical dysplasia in the same area. Interictal EEG dipoles were concentrated in the right mesial occipital lobe. Both interictal and ictal MEG dipoles were concentrated in the right mesial occipital lobe, which corresponded well with neuroimaging data and his clinical features. Case 2 is a 5-year-old female suffering with simple partial seizures with left-side facial twitching. Interictal EEG dipoles were located in her left motor area, the pre-sylvian fissure, close to the location of the interictal MEG-estimated dipoles. Ictal EEGs showed no remarkable changes associated with her clinical manifestations. However, ictal MEG showed high-voltage slow waves over her left hemisphere, and ictal MEG iso-contour maps revealed a clear dipolar pattern, which suggested that the MEG dipole was located in the area of the sylvian fissure. Ictal SPECT revealed hyperperfusion areas around the left sylvian fissure. Conclusion: Ictal MEG is useful for determining the precise location of epileptic focus in patients with motionless seizures, including children.     

Partial seizures manifesting as apnea only in an adult

PURPOSE: Although several cases of apneic seizures have been reported in neonates, epileptic seizures presenting as apnea only in adults are very rare. We present a case report of a 19-year-old man with viral encephalitis and frequent episodes of apneic seizures. METHODS: Prolonged electroencephalograms (EEGs), respiratory monitorings, and imaging including ictal-interictal subtraction single photon emission computed tomography (SPECT) coregistered with magnetic resonance imaging (MRI) were performed. RESULTS: Ictal EEGs recorded during apneic episodes showed repetitive sharp waves or rhythmic theta activity arising from the left or right independent bitemporal region. Ictal SPECT was performed during one episode of apnea that showed ictal EEG discharges arising from the left posterior temporal area. Ictal-interictal subtraction SPECT coregistered with MRI revealed that the seizures originated from the left, posterior, midlateral temporal cortex. CONCLUSIONS: Previous studies with ictal EEG or brain stimulation suggest that apneic seizures might be mediated through the limbic and associated cortical systems. Our study reports on a very rare case of partial seizures with apnea only in an adult patient and is supported by ictal EEG and ictal-interictal subtraction SPECT coregistered with MRI.     

Ictal Video-EEG Recording of Three Partial Seizures in a Patient with the Benign Infantile Convulsions Associated with Mild Gastroenteritis

PURPOSE: In infants, benign convulsions can be triggered by febrile illness or mild diarrhea such as Rotavirus gastroenteritis. The triggering mechanism of these convulsions is still unknown. In spite of several reports concerning clinical features, the ictal EEG recordings were rarely analyzed by a video-EEG monitoring system. To reveal a clue for the triggering mechanism of these convulsions, we analyzed the correlation of clinical manifestations and the EEG discharges during the ictal events and compared with previous reports. METHODS: The ictal EEG of a cluster of three afebrile convulsions associated with mild gastroenteritis was recorded by an EEG closed-circuit TV (EEG-CCTV) monitoring system in a 6-month-old healthy female infant. RESULTS: All seizures began as complex partial seizures (CPSs), which exhibited a motionless stare with or without leftward deviation of both eyes, and evolved to secondarily generalized tonic-clonic seizures (SGTCSs) for approximately 90 s. Each of three ictal discharges began from the right occipital, right centroparietotemporal, and left occipital regions, respectively. CONCLUSIONS: Although initiating sites of ictal discharges of benign infantile convulsions associated with mild gastroenteritis (BICE) were previously reported to be variable among patients, these results indicated that those differ among seizures even in a same infant.     

Seizures manifesting as an urge to defecate, with an ictal discharge in the right hemisphere

We report a 47-year-old right-handed woman with simple partial seizures manifesting in part as an urge to defecate shortly after a visual distortion. On at least one occasion, this was followed by head deviation to the left and a secondarily generalized tonic-clonic seizure. The ictal electroencephalogram lateralized to the right hemisphere, suggesting that the ictal urge to defecate may lateralize to this side.     

Episodische Epilepsie

We report two patients with episodic seizure clustering with a delayed diagnosis of epilepsy. Patient 1: a 68-year-old woman had a four year history of clusters of complex partial seizures. Repetitive seizures manifested almost continuously for 24 hours every 30 or 31 days. Interictal electro-encephalograms (EEG) showed a normal α-rhythm. An ictal EEG demonstrated rhythmic theta-activitiy over the whole left temporal lobe. Patient 2: a 30-year old man had a four year history of clusters of complex partial seizures once a year. Seizures manifested repetitively over a period of up to four days once a year. Interictal EEGs were normal. MRI showed right hippocampal sclerosis. Ictal EEG demonstrated rhythmic right temporal theta activity.¶???In conclusion, normal interictal investigations in patients with infrequent epileptic seizures or clusters of seizures do not exclude a diagnosis of epilepsy.     

Non-convulsive status epilepticus presenting with Wernicke's aphasia

Ictal aphasia in adults is a rare phenomenon. Most reported cases manifest with non-fluent (Broca) aphasia. Ictal fluent (Wernicke) aphasia is less common. We report a 47-year-old, right-handed woman that presented with recurrent episodes of non-convulsive seizures in the form of Wernicke's aphasia for 2 weeks. An MRI of the brain showed an old cerebral infarction in the left parieto-occipital area. Scalp EEG revealed continuous periodic sharp waves at the left temporal regions with diffusion to the whole left hemisphere and at occasions to the right. This is followed by variable periods of post ictal slowing. Recurrence of the described ictal pattern was noted. Management of status epilepticus was started in the form of intravenous diazepam and a loading dose of phenytoin and phenobarbitone. After treatment, she improved clinically and the EEG improved with disappearance of the left temporal ictal rhythm and normalization of the EEG background. Thus, establishing the diagnosis of non-convulsive partial status epilepticus manifesting as ictal aphasia.     

Contraversive seizures in occipital epilepsy: case report and review of the literature

We studied a man with repeated seizures characterized by deviation of the head and eyes to the left. A right occipital focus was demonstrated in the EEG. Adversion is usually associated with frontal or temporal lobe seizures; the direction of adversion is equally likely to be ipsilateral as contralateral to the ictal focus. In all reports of occipital epilepsy, the ictal focus has been contralateral to the direction of head turning.     

Ictal paralysis with tonic eye gazing mimicking a pontine infarction

PURPOSE: Concomitant positive and negative motor phenomena in a single seizure have not been reported before. METHOD: We used an extensive history review, neurological examination, EEG, MRI and SPECT study to demonstrate a rare combination of motor presentations as an ictal phenomenon. RESULT: A 64-year-old male was brought to the emergency room with dizziness, progressive drowsiness and left hemiparesis. A spontaneous eye deviation to the left side with nystagmus was observed. A right pontine lesion was tentatively diagnosed. However, a focal motor seizure of the patient's left face and limbs occurred 3.5h later. A brain MRI revealed a high signal in the right amygdala, hippocampus and thalamus, instead of the pons. An EEG showed periodic epileptic discharges in the right posterior temporal parietal region. Regional hyperperfusion was found by brain SPECT. The level of consciousness improved dramatically after adequate phenytoin treatment. CONCLUSION: A posterior temporal-parietal seizure can present with a prolonged ictal paralysis, a positive ocular nystagmoid deviation and an altered level of consciousness. The EEG is essential for a correct diagnosis, especially with a negative or an unexplainable MRI study. The SPECT has an additional role for the differential diagnosis.     

Musicogenic epilepsy with ictal single photon emission computed tomography (SPECT): could these cases contribute to our knowledge of music processing?

Musicogenic epilepsy has a strong correlation with the temporal lobe with a right-sided preponderance. We report the case of a 48-year-old woman whose seizures began at the age of 32 years. Her prenatal, natal and childhood histories were unremarkable and her family history was negative for epilepsy. She had typical complex partial seizures with chewing automatisms. Cranial computed tomography, magnetic resonance imaging (MRI) and interictal SPECT showed no abnormality. Interictal EEG showed paroxysmal bitemporal sharp wave discharges predominant on the right side. Ictal EEG showed a combination of high voltage sharp and slow sharp waves and spikes that originated from the right temporal leads and then became generalized. Ictal activity on EEG started 4-5 min after the music stimulus. For the ictal SPECT study, i.v. injection of 20 mCi of HMPAO was administered approximately 30 s after the ictal activity started. Ictal SPECT demonstrated a right anterior and mesial temporal hyperperfusion. These results seem to support the dominant role of the right temporal lobe and the possible relation of mesial temporal structures to the affective content of music in musicogenic epilepsy.     

Epileptic kinetopsia: ictal illusory motion perception

A 38-year-old woman with a right posterior temporo-occipital brain tumor developed partial seizures with illusory motion perception of environmental objects going from the center to the periphery within her left visual field. Subdural EEG recordings during visual seizures revealed onsets in the right temporo-parieto-occipital junction. Her ictal visual distortion was probably caused by activation of V5, an area involved in motion perception. Given that the tumor location corresponds with the ictal onset in the V5 area, and the semiology of her seizures, this case supports that epileptic dysfunction in V5 can cause illusions of visual motion.     

A case with frontal lobe epilepsy presenting with absence seizures as cardinal manifestation: ictal EEG findings]

We report here a 9-year-old boy presenting with absence and complex partial seizures. Absence seizures occurred several times a day, with sudden arrest of speech and gesture, alteration of consciousness, myoclonus of unilateral or bilateral angles of the mouth, occasional simple automatism and brisk recovery of consciousness. Complex partial seizures occurred once to three times a month with loss of consciousness, salivation, deviation of the head and eyes toward the left, elevation of upper limbs and tonic convulsion of the left upper and lower limbs. Interictal EEG showed right frontal pole-dominant high-voltage slow waves or spike-and-waves. Ictal simultaneous video-EEG recordings of absence seizures revealed a frontal dominant 3-3.5 Hz spike-wave burst lasting several seconds. A partial seizure never preceded the absence seizure. Transverse topographical analysis revealed that the first spike component of the spike-wave burst of absence seizure always showed phase reversal on the right anterior temporal electrode. The following ones, however, showed phase reversal on the left anterior temporal electrode. Ictal EEG of the complex partial seizure could not be detected because it rarely occurred. There was no abnormal finding on brain MRI. Interictal single photon emission tomography (SPECT) indicated hypoperfusion of the dorsal and medial cortex of the right middle frontal lobe. Interictal positron emission tomography (PET) also indicated hypometabolic areas in the dorsal and medial cortex of the right frontal lobe, together with those in the right temporal and parietal cortex. EEG evolution and neuroimaging studies suggested that the epileptic focus of the absence seizure might have originated at the dorsal cortex of the right middle frontal lobe and immediately spread to the medial cortex. Both the seizures were well controlled by the combination of phenytoin and high dose sodium valproate.     

Bathing epilepsy: Video/EEG recording and literature review

We report a 3-year-old boy with bathing epilepsy clinically manifesting as complex partial seizures precipitated by immersion in warm water during bathing and showering. Two seizures were recorded on video. During one, a simultaneous ictal EEG recording revealed an ictal pattern over the left hemisphere. Warm water was demonstrated to be the specific precipitating stimulus; hot dry towels and cold water did not provoke seizures or EEG abnormalities.     

Ictal asomatognosia due to dominant superior parietal cortical dysplasia

We report a 23-year-old man with left dominant parietal cortical dysplasia manifesting as ictal asomatognosia. The man had experienced seizures, during which he underwent ictal asomatognosia as a feeling of loss of his right extremities. Scalp electroencephalography (EEG) showed interictal discharges in the left parietal region of his brain. Magnetic resonance fluid-attenuated inversion recovery (FLAIR) imaging revealed a hyperintense lesion in the left superior parietal lobule. A [¹²³I]-iomazenil (IMZ) single-photon-emission CT scan demonstrated an area of low IMZ binding coincident with the lesion observed in the MRI scan. Invasive EEG monitoring showed ictal discharges in the cortex posterior to the postcentral sulcus. High-frequency electrical stimulation of the same area of the cortex also induced asomatognosia of the patient’s right forearm. We performed a corticectomy of the anterior part of the superior parietal lobule, which resulted in no new neurological deficits. The seizures disappeared after surgery with the maintenance of preoperative medication. Therefore, the anterior part of the superior parietal lobule may be a symptomatogenic zone for ictal asomatognosia.     

A case of parietal lobe epilepsy with ictal laughter

We report here about an 8-year-old boy with parietal lobe epilepsy (PLE) and ictal laughter. At the age of 6, he began to experience drop seizures, followed by sensory fits. Interictal EEG showed frequent spikes at C3, C4, P3 and Cz. Despite treatment with antiepileptic drugs, he often fell down in seizures after feeling abnormal sensations in the right shoulder. On ictal video EEG at the age of 7 years, (1) he became motionless and complained of fear and pain in the right hand, (2) he had clonic seizures of the right upper limb and fell down to his left, (3) he laughed though he did not feel funny. Ictal EEG showed spikes which originated in Pz and then were generalized. In many of the previously reported cases, ictal laughter is associated with hypothalamic hamartomas, infantile spasms,. complex partial seizures of frontal, temporal, or parietal origin. We diagnosed the present case as having PLE. However, other localization could not be roled out because the spikes were generalized quickly. To date, there are two reported cases of ictal laughter with PLE, but ictal EEG is lacking in these patients. Ictal laughter is rare in non-lesional cryptogenic PLE, but it may imply PLE's pathogenesis.     

Epileptic seizures and electroencephalographic evolution in genetic leukodystrophies.

The purpose of this study is to explore and compare epileptic seizures and EEG evolution in the various types of genetic leukodystrophy (GL). The authors reviewed the medical records and analyzed 69 serial EEGs in 27 patients with GLs: 13 with late infantile metachromatic leukodystrophy, one with juvenile metachromatic leukodystrophy, one with globoid cell leukodystrophy, six with X-linked childhood adrenoleukodystrophy, one with neonatal adrenoleukodystrophy, four with classic Pelizaeus-Merzbacher disease (PMD), and 1 with connatal Pelizaeus-Merzbacher disease. The diagnoses were made by biochemical and molecular studies. Two or more EEG studies with both awake and sleep traces were recorded during the varying clinical stages for each patient. At the beginning of the GLs, the EEGs were normal or showed mild slowing of background activity. Clinical seizures, mainly of focal origin, with progressive slowing and paroxysmal discharges on EEGs, usually appeared during the later stages of metachromatic leukodystrophy, X-linked childhood adrenoleukodystrophy, and classic Pelizaeus-Merzbacher disease. However, intractable seizures, mainly generalized in nature, and more severe slowing and abundant paroxysmal discharges on EEGs, with commensurate neurologic deterioration, were observed during the earlier course of globoid cell leukodystrophy, neonatal adrenoleukodystrophy, and connatal Pelizaeus-Merzbacher disease. These results indicate that GLs involve not only white matter, but gray matter as well. In all types of GL, there is good correlation between the severity of EEG changes, the severity of the diseases, and the clinical state of the patient.     

Focal Cerebral Magnetic Resonance Changes Associated with Partial Status Epilepticus

Summary: We report 2 patients with transient abnormalities on magnetic resonance imaging (MRI) associated with partial status epilepticus (SE). A man with a 4-month history of partial seizures had complex partial SE for 9 days, with left temporal maximum on ictal EEG. Left temporal lobe T₂ signal was increased on MRI during SE, but cerebral MRI was normal 9 weeks later. A woman with "cryptogenic" temporal lobe epilepsy for 16 years had complex partial SE for 1 week, with right temporal maximum on ictal EEG. T₂ Signal was increased over the entire right temporal lobe, extending into the insula, without mass effect, on MRI 1 month after SE ended. Repeat MRI 1 month later showed marked decrease in volume of increased T₂ intensity, without gadolinium enhancement, but with mild mass effect over the right anteroinferomesial temporal areas. A gemistocytic astrocytoma was resected. Focal cerebral MRI abnormalities consistent with cerebral edema may be due to partial SE but also may indicate underlying glioma, even in long-standing partial epilepsy. Focal structural imaging changes consistent with neoplasm should be followed to full resolution after partial SE.     

Writing epilepsy: a neurophysiological, neuropsychological and neuroimaging study

Writing epilepsy is a rare reflex syndrome in which seizures are triggered by writing. We describe a 33-year-old, right-handed man, with a history of juvenile absence epilepsy in remission and a family history of epilepsy, in whom myoclonic jerks precipitated exclusively by writing started at the age of 30. Intensive video/EEG monitoring during neuropsychological tests revealed, at about 1 minute after starting to write, a dystonic posture, followed by myoclonic jerks involving the right hand that shortly after became generalized. Concomitantly, the ictal EEG documented generalized hypersynchronous polyspike-wave discharges, maximal over the right parietocentral area. SPECT revealed an ictal hyperperfusion and interictal hypoperfusion over right parietofrontal regions, and fMRI showed extensive and intense left frontal, supplementary motor area activation, induced by writing. This case study provides some evidence supporting the hypothesis that the mechanism underlying writing-triggered seizures may be a generalized seizure process, with a focal cortical trigger zone, presumed to be the left frontal lobe as suggested by clinical and fMRI data. A relevant role played by the right hemisphere (right parietofrontal region) is postulated in the full-blown expression of reflex epileptogenesis, as supported by EEG and SPECT findings.     

Episodic nausea and abdominal sensations as sole manifestations of simple partial seizures

In adults, seizures manifesting with abdominal complaints are usually associated with complex partial or secondary generalized seizures. Also, seizure periodicity is not expected in postmenopausal women. We encountered a 72-year-old woman with episodic nausea and abdominal pain that usually occurred with predictable regularity. When symptoms persisted after extensive gastrointestinal investigations and cholecystectomy, she was referred to us and the diagnosis of simple partial seizures was suspected. Both EEG and brain MRI were normal. The diagnosis was established by video/EEG monitoring, which recorded several typical clinical events associated with right temporal ictal discharges. Because treatment with several antiepileptic medications caused intolerable side effects, the patient is now maintained on a low dose of Lamotrigine, which reduced seizure frequency and severity. This patient demonstrates that `abdominal` complaints, although rare, may be the sole manifestation of simple partial seizures. Unless considered in the differential diagnosis, the patient may undergo unnecessary and potentially harmful procedures.     

Epileptic ictal strabismus: a case report and review of the literature

Ictal strabismus, sometimes associated with epileptic nystagmus, is an extremely rare epileptic phenomenon, suggestive of cortical involvement in monocular eye movement control. We describe a patient with ictal disconjugate contraversive horizontal eye deviation of cortical origin as the main clinical feature of a focal seizure. A 17‐year‐old, previously healthy woman had a seizure characterized by initial rightward conjugate eye deviation, followed by convergent strabismus due to adduction of the right eye towards the nose without conjugate left eye abduction (esotropia), forced leftward head deviation with impaired awareness, and subsequent evolution into a bilateral tonic‐clonic seizure. Postictal and interictal neurological status were unremarkable; more specifically, neuro‐ophthalmological examination revealed no nystagmus or altered eye motility. Ictal EEG showed a rhythmic theta activity over the right posterior temporal region, involving fronto‐central regions when strabismus appeared. MRI showed cortical dysplasia in the right temporal lobe. Due to the low spatial resolution of scalp EEG, we could not identify with precision the symptomatogenic zone underlying ictal strabismus. However, the concomitant appearance of rhythmic theta activity over the right fronto‐central region and the leftward head version with MRI perfusion sequences, showing cerebral blood flow increase in the right frontal eye field area, suggest involvement of the right frontal lobe. [Published with video sequence on www.epilepticdisorders.com ].