An epilepsy case presenting temporal aphonia and prosodic deficit with left hemiparesis]

A 53-year-old right-handed woman was admitted to a hospital with a seizure, exhibiting loss of consciousness and paroxysmal myoclonic movement. MRI showed no abnormalities of her brain. Electroencephalography revealed sporadic sharp and slow wave complexes, starting from the right central region. Cerebral perfusion SPECT revealed increased blood supply in the right hemisphere. She received diagnosis of epilepsy and was treated with phenytoin. After she regained her consciousness, she had no voice and presented with left hemiparesis which was interpreted to be Todd's palsy. She gradually recovered in using her voice, to full normalization of affective prosody in 11 days. The left hemiparesis recovered first in the upper and later in the lower extremities. Her symptoms could be interpreted to be vocal and prosodic disturbance, but not aphasia, because she could understand speach and communicate with writing. The lesions responsible for prosodic deficit are still controversial. The symptoms observed and the findings obtained may indicate that the deficiency of the affective prosody may be caused by the dysfunction of the medial surface of the non-dominant frontal lobe in this case.     

Rare combination of gelastic epilepsy, agenesis of the corpus callosum, and hamartoma

Gelastic seizures are rare and are associated with different conditions, but mainly with hypothalamic hamartoma. We report on a boy who presented with mental retardation, aggressive behavior, and generalized tonic-clonic and gelastic seizures. Cranial imaging studies revealed a very rare combination of hypothalamic hamartoma and agenesis of the corpus callosum, which was only reported once previously. His seizure activities demonstrated a modest response to anticonvulsants.     

An operated case of bitemporal lobe epilepsy associated with old infarction in the left occipital lobe]

A 15-year-old female, who presented with bitemporal lobe epilepsy associated with old infarction in the left occipital lobe, was reported. MRI with fluid attenuated inversion recovery sequence demonstrates cortical atrophy with hyperintensity of the white matter in the left occipital lobe as well as volume loss and hyperintensity of the left hippocampus. Interictal positron emission tomography with [18F]fluorodeoxy glucose (FDG-PET) and single photon emission computed tomography with technetium-99m-ethyl-cysteinate dimer indicate hypometabolism and hypoperfusion in the left occipital lobe and the left temporal lobe, respectively. Scalp recorded EEG did not lateralize the side of the epileptogenic zone. Chronic subdural electrode recording demonstrated that the ictal onset zones were located in the bilateral side of the temporal lobe. Eighty-nine percent of 19 spontaneous seizures were left sided onset. The anterior temporal lobectomy with hippocampectomy was performed for the left side. Although temporal lobe epilepsy is sometimes a bilateral disease, unilateral lobectomy for a strong predominant side, based on the MRI and FDG-PET findings, is effective for some patients.     

Post-ictal gustatory hallucination, sleep related microspikes and glioma of the sylvian region: report of a case

A 32 year old man affected by grade 1 astrocytoma of the right sylvian region and epileptic seizures, presented gustatory hallucinations as a post-ictal symptom. Spikes of very low voltage during sleep were the only epileptic abnormalities found on his EEG. It is suggested that post-ictal as well as ictal gustatory hallucinations may be a localizing symptom of glioma involving the sylvian region. Furthermore, if systematically searched for, sleep related microspikes may become an EEG sign useful for the diagnosis of brain tumour in patients with epileptic seizures and no epileptic discharges on the awake EEG.     

Non-verbal auditory agnosia with EEG abnormalities and epilepsy; an unusual case of Landau-Kleffner syndrome]

A case of unusual Landau-Kleffner syndrome was reported. She was an 8 year-old girl and showed non-verbal agnosia, diffuse EEG abnormalities and convulsions. Her responses to both verbal and non-verbal sounds remained inconsistent and unstable. When a continuous spike-wave complexes on EEG was detected, she paid no attention to any sound in spite of her fair consciousness. Auditory brainstem response and magnetic resonance imaging of her brain were normal. Auditory agnosia was correlated well with EEG abnormalities, and valproic acid and clonazepam were effective for EEG improvement. After the EEG improvement, clinical responses to sounds recovered well; firstly she could pay attention to sounds and then she could distinguish between verbal and non-verbal sounds. Finally, she could speak a few words after the learning letters.     

Ictal visual hallucination intermittent photic stimulation: using evaluation of the clinical findings, ictal EEG, ictal SPECT, and rCBF]

A 43-year-old, right-handed woman experienced right hand paresthesias and a visual field abnormality. We attributed her symptoms to psychiatric abnormalities, due to the presence of delusions and auditory hallucinations. Upon photostimulation, she experienced left visual field hallucinations and demonstrated slow waves on the right parieto-occipital regions. The clinical and electro-encephalographic findings suggested that these episodes were epileptic seizures originating from the right occipital region. Ictal fear appeared at the end of the seizure, reflecting the spread of seizure activity to the mesial temporal region. Ictal SPECT images showed hyper-perfusion in the right occipital region and left cerebellar cortex. rCBF in the occipital lobe was significantly asymmetrical. When we encounter an epileptic patient with psychosis who has a visual hallucination, we should consider the possibility of epileptic seizure originating from the occipital lobe.     

Interictal EEG spikes identify the region of electrographic seizure onset in some,but not all,pediatric epilepsy patients

Purpose : The role of sharps and spikes, interictal epileptiform discharges (IEDs), in guiding epilepsy surgery in children remains controversial, particularly with intracranial electroencephalography (IEEG). Although ictal recording is the mainstay of localizing epileptic networks for surgical resection, current practice dictates removing regions generating frequent IEDs if they are near the ictal onset zone. Indeed, past studies suggest an inconsistent relationship between IED and seizure‐onset location, although these studies were based upon relatively short EEG epochs. Methods : We employ a previously validated, computerized spike detector to measure and localize IED activity over prolonged, representative segments of IEEG recorded from 19 children with intractable, mostly extratemporal lobe epilepsy. Approximately 8 h of IEEG, randomly selected 30‐min segments of continuous interictal IEEG per patient, were analyzed over all intracranial electrode contacts. Results : When spike frequency was averaged over the 16‐time segments, electrodes with the highest mean spike frequency were found to be within the seizure‐onset region in 11 of 19 patients. There was significant variability between individual 30‐min segments in these patients, indicating that large statistical samples of interictal activity were required for improved localization. Low‐voltage fast EEG at seizure onset was the only clinical factor predicting IED localization to the seizure‐onset region. Conclusions : Our data suggest that automated IED detection over multiple representative samples of IEEG may be of utility in planning epilepsy surgery for children with intractable epilepsy. Further research is required to better determine which patients may benefit from this technique a priori.     

An unusual cause of apparent epilepsy: ECG and EEG findings in a case of Jervell Lange-Neilson syndrome.

A case is presented of apparent epilepsy which proved to be due to recurrent ventricular tachyarrhythmias (torsade de pointe). The relationship between the cardiac arrhythmia and changes in the electroencephalograph is recorded and analysed. This is probably an example of the 'Jervell Lange-Neilson' syndrome of cardiac arrhythmias which may produce ictal episodes, prolongation of the QTc interval of the ECG, and sensori-neural deafness. The features of the syndrome, its pathology and treatment, and its relevance to the mangement of epilepsy are discussed.     

Seizure frequency, patient-perceived seizure severity and the psychosocial consequences of intractable epilepsy.

It is generally recognised that the assessment of treatment effects in epilepsy using seizure frequency as the only outcome measure may lack sensitivity. A patient-based seizure severity scale has been developed and initial results confirm its reliability and validity. As part of the further development of this scale it is important to explore the relationship between seizure severity, seizure frequency and the psychosocial consequences of intractable epilepsy. One hundred patients with medically refractory partial seizures completed a quality of life questionnaire including measures of physical (seizure severity and frequency), social and psychological well-being (anxiety, depression, self-esteem, locus of control and happiness). Multivariate analysis demonstrated that individual psychological variables were best predicted by other psychological variables. However, when these were removed from analysis, seizure severity was the most significant predictor of self-esteem (P = 0.005), locus of control P = 0.039) and anxiety (P = 0.048). Seizure frequency did not contribute significantly to the variance of any of the psychological factors. These results highlight the importance of considering seizure severity when assessing treatment effects in epilepsy and provide further evidence for the construct validity of a novel patient-based seizure severity scale.     

A case of the familial essential myoclonus and epilepsy presenting behavioral arrest]

A 75-year-old Japanese man who had a past history of epilepsy was admitted because of rhythmical myoclonus of upper and lower extremities. His mother and son also had both epilepsy and myoclonus. On neurological examination, he was alert and oriented without dementia. There was no abnormal finding except for the myoclonic jerks appearing in his upper and lower extremities. The myoclonic jerks appeared at rest and worsened during maintaining posture. Results of laboratory tests were all within normal levels. Brain MRIs were normal, and his EEGs showed diffuse fast wave activities without paroxysmal discharges. Surface EMG studies revealed irregular myoclonic discharges at the frequency of 6-20 Hz. Electrophysiologically, he had a giant somatosensory-evoked potentials, an enhanced long-loop reflex(C-reflex) and cortical spikes preceding the myoclonic jerks, suggesting that his myoclonus was of cortical origin. Based on the cortical myoclonus, episodes of epilepsy and familiarity, he was diagnosed as having familial essential myoclonus and epilepsy(FEME). In addition, he periodically became a motionless state characterized by sudden appearance of transient akinesia with generalized stiffness of all of his limbs. The transient motionless state usually appeared following mental stress or sudden sensory stimuli such as loud sound. It persisted for about 10-20 min and ceased spontaneously. Since EEGs recorded during this akinetorigid state showed no paroxysmal discharges, it was considered not to be epileptic. However, after the administration of valproate, his cortical myoclonus improved, and the transient akinetorigid state also disappeared simultaneously. From symptomatic point of views, this generalized motionless state with stiffness of limbs may resemble to freeze behavior of wild animals suddenly confronted with sudden strong stimuli. From symptomatological as well as genetic aspects, FEME has been suggested to be heterogeneous. We propose that this unique akinetorigid attack might be a new clinical phenotype of FEME.     

An unusual posterior mediastinal lipoblastoma with spinal epidural extension presenting as a painful suprascapular swelling: case report and a brief review of the literature.

Lipoblastoma is a rare benign pediatric neoplasm of fetal-embryonal fat with little risk of recurrence following total microsurgical excision, but it may progress to local invasion or infiltration if not treated surgically. No adjuvant therapies are usually necessary once the tumor is excised. It is best diagnosed on histopathological studies following excision. An unusual posterior mediastinal lipoblastoma in a 2-year-old Omani girl with spinal epidural extension clinically manifested as a progressive painful suprascapular swelling is reported.It was initially construed to be a benign lipoma, but progressively increasing pain and mild imbalance whilst walking with a tendency to fall on the right side prompted neurosurgical referral and eventual total excision without any added morbidity. Interesting clinical and neuroimaging (CT & MRI) findings are presented and discussed, with a brief review of the literature.     

A case of progressive myoclonus epilepsy with the first and second branchial syndrome]

A 22-year-old woman with progressive myoclonus epilepsy associated with the first and second branchial syndrome is described. Clinical features included generalized convulsive seizure, myoclonus, cerebellar ataxia and intellectual deterioration with micrognathia and malformation of auricles. She was initially suspected of mitochondrial encephalomyopathy, but the analysis of muscle biopsy and mitochondrial enzyme activities was negative. Her micrognathia and malformation of auricles were diagnosed as the first and second branchial syndrome. The case of progressive myoclonus epilepsy associated with this syndrome has never been reported, and the relationship between them remains unknown.