Congruence of the topography of intracranial calcifications and epileptic foci.

Nodular intracranial calcifications (NIC) are frequent findings in CT scans of epileptic patients in countries where granulomatous central nervous disease such as neurocysticercosis is endemic. In 34 consecutive epileptic patients with NIC submitted to EEG, CT and CSF analysis, the correlation between the electroclinical localization of the focus and the topography of the NIC was studied. Twenty-nine patients had partial (Group I) and 5 had primarily generalized seizures (Group II). Twenty group I and 1 group II patients showed abnormal EEGs. CSF abnormalities consisted of increased protein content (n = 3) and positive Weinberg's reaction (n = 2). In 2 cases, viable neurocysticercotic vesicles were seen. Twenty-one patients had single NICs. No correlation could be established in group II patients. Within group I, 15 patients had a positive and 14 a negative correlation. Sixty-six percent of the patients with single NICs had negative correlations. These findings strongly suggest that the calcifications themselves are not the epileptogenic lesions in at least 50% of the studied cases.

Congruence of the topography of intracranial calcifications and epileptic foci

Calcificaçöes nodulares intracranianas (CIN) representam achado frequente em pacientes epilépticos de países onde doenças granulomatosas do SNC(p.e.: neurocisticercose) säo endêmicas. Trinta e quatro pacientes epilépticos consecutivos com CINs foram submetidos a exame de EEG, TC e LCR. A correlaçäo entre a topografia do foco e das calcificaçöes foi estudada em cada caso. Vinte e nove pacientes tinham crises parciais (Grupo I) e 5 crises primariamente generalizadas (Grupo II). Vinte pacientes do Grupo I e 1 do grupo II tinham EEGs anormais. Hiperproteinorraquia (n=3) e reaçäo de Weinberg positiva foram as alteraçöes encontradas no LCR. Em 2 casos, vesículas neurocisticercóticas íntegras foram vistas na tomografia. Vinte e um pacientes possuíam CIN única. Näo foi possível realizar a correlaçäo clínica nos pacientes do Grupo II. No grupo I, 15 pacientes apresentaram correlaçäo positiva e 14 negativa. Sessenta e quatro por cento dos pacientes com calcificaçäo única apresentaram correlaçäo negativa. Estes achados sugerem fortemente que as calcificaçöes näo säo epileptogênicas em ao menos 50 por cento dos casos estudados

MRI and brain spect findings in patients with unilateral temporal lobe epilepsy and normal CT scan.

26 patients with temporal lobe epilepsy clinically documented by several abnormal interictal surface EEGs with typical unitemporal epileptiform activity and a normal CT scan were studied. Interictal 99mTC HMPAO brain SPECT and MRI were performed in all subjects. Abnormalities were shown in 61.5% of MRI (n = 16) and 65.4% of SPECT (n = 17). Hippocampal atrophy associated to a high signal on T2-weighted MRI slices suggesting mesial temporal sclerosis was the main finding (n = 12; 75% of abnormal MRI). MRI correlated well to surface EEG in 50% (n = 13). There was also a good correlation between MRI and SPECT in 30.7% (n = 8). SPECT and EEG were in agreement in 57.7% (n = 15). MRI, SPECT and EEG were congruent in 26.9% (n = 7). These results support the usefulness of interictal brain SPECT and MRI in detecting lateralized abnormalities in temporal lobe epilepsy. On the other hand, in two cases, interictal SPECT correlated poorly with surface EEG. This functional method should not be used isolately in the detection of temporal lobe foci. MRI is more useful than CT as a neuroimaging technique in temporal lobe epilepsy. It may detect small structural lesions and mesial temporal lobe sclerosis which are not easily seen with traditional CT scanning.

MRI and brain SPECT findings in patients with unilateral temporal lobe epilepsy and normal CT scan

Foram estudados 26 pacientes com epilepsia do lobo temporal clinicamente documentada por vários EEGs com atividade epileptiforme intercrítica e TC de crânio normal SPECT com TC HMPAO e ressonância magnética (RM) foram realizados em todos os casos 61,5 por cento (n=17) dos SPECTs revelaron algum tipo de anormalidade. Atrofia hipocampal, nas imagens enfatizadas em T1 e presença de hipersinal nas imagens enfatizadas em T2, sugerindo esclerose mesial temporal, foram o principal achado (n=12,75 por cento das RM anormais). RM correlacionou-se ao ECG em 50 por cento (n=13). Concordância entre RM e SPECT ocorreu em 30.7 por cento (n=8) e entre SPECT e EEG em 57.7 por cento (n=15), RM, SPECT e EEG foram congruentes em 26.9 por cento (n=7). Estes resultados refletem o valor do SPECT intercrítico e RM na detecçäo e lateralizaçäo de anormalidades em epilepsia do lobo temporal. Por outro lado, em dois casos, o SPECT correlacionou-se mal com o EEG. Este método funcional näo deve ser utilizado isoladamente na detecçäo de focos temporais. RM é mais útil do que a TC como recurso de neuroimagem na epilepsia do lobo temporal. Ela pode detectar pequenas lesöes estruturais e esclerose mesial temporal, näo facilmente identificáveis pela TC

Eating epilepsy.

Eating epilepsy is a rare type of reflex epilepsy. A 24 years-old male with eating reflex complex partial seizures was submitted to clinical, neurological, neuroradiological and EEG studies. Neurologic and CT examinations were normal. EEG recordings including video-EEG monitoring during meals disclosed focal abnormalities related to both temporal lobes prevailing at the left side and secondary bilateral synchrony mainly in more anterior regions. Ictal findings were similar to the interictal secondary bilateral synchrony except for its longer duration. PB, VPA and DPH monotherapies were ineffective. High dose CBZ monotherapy yielded good but incomplete seizure control. Since a big number of precipitants could be involved, no specific physiopathological basis could be established.

Eating epilepsy

A epilepsia reflexa ao comer é uma rara de epilepsia reflexa. Um paciente de 24 anos com crises parciais complexas reflexas ao comer foi submetido a avaliaçöes clínica, neurológica neurorradiológica e eletrencefalográfica. O exame neurológico e a tomografia de crânio foram normais. Registros de EEG, incluindo menitorizaçäo vídeo-EEG durante a refeiçäo, mostraram presença de anormalidades focais relacionadas a ambos os lobos temporais, predominando à esquerda, e sincronia bilateral secundária, predominando em regiöes anteriores. Os achados ictais säo semelhantes à sincronia bilateral secundária interictal exceto por sua maior duraçäo. Monoterapias com PB, DPH e VPA näo surtiram efeito. Monoterapia com altas doses de CBZ trouxe bons resultados porém com controle incompleto das crises. Como grande número de fatores precipitantes estava potencialmente envolvido, näo foi possível determinar com precisäo a base fisiopatológica das crises reflexas neste caso

Mental deterioration in Lafora's disease.

Lafora's disease is included among the progressive myoclonic epilepsies. Despite the fact that dementia is a constant finding in this disease only a few papers have studied the timing of mental deterioration. We have performed wide neuropsychological testing in two cases early diagnosed as Lafora disease. The initial neuropsychological testing was carried out by the time there were no complaints of mental deterioration in both cases. In the first case consecutive neuropsychological testing demonstrated the rapidly progressive dementia. All neuropsychological testings in these cases showed severe impairment of right parietal lobe functions. Higher cortical functions related to language and intellectual processes were best preserved in both cases. The functions related to constructional praxis, memory and abstract concepts and processes were severely impaired. Our data suggest that mental deterioration is an early manifestation in Lafora disease, even by the time normal social life is not yet disturbed. Dominant hemisphere cognitive functions have been less impaired than the non-dominant ones. How a diffuse illness such as Lafora disease can cause such an asymmetrical higher cortical function deficit is not yet clear.

Non-epileptic myoclonus and mitochondrial encephalomyopathy.

Two brothers presented to us with a progressive myoclonic syndrome with slight cerebellar symptoms. Neurological examination disclosed moderate cerebellar signs and pale optic discs; asymmetric, asynchronous and arrhythmic myoclonus, an arrthesthesic deficit and no muscular weakness. EEG background activity was moderately slow with no irritative discharges. CT was normal in both cases. Intermittent photic stimulation increased the frequency of the myoclonic jerks, which became bilateral and synchronous, progressing to a generalized tonic-clonic seizure. EPs and MRI in one case were normal. Anticonvulsant drugs were ineffective. The diagnosis of mitochondrial encephalomyopathy was based on the finding, in muscle specimens, of thickened basement membranes with myofibrillary degeneration and increased number of mitochondria peripherally distributed and with a dense granular matrix and some vacuoles. The clinical and EEG data suggest a subcortical origin for this type of myoclonic syndrome.

Non-epileptic myclonus and mitochondrial encephalomyopathy

Foram examinados dois irmäos portadores de uma síndrome miclónica progressiva comn discretos sintomas cerebelares. O exame neurológico mostrava sinais cerebelares moderados e papilas pálidas; mioclonais assíncronas, arrítmicas e assimétricas, um déficit artrestésico e ausência de fraqueza muscular. A atividade de base do EEG era moderadamente lenta e sem atividade irritativa. A TC era normal em ambos os casos. A estimulaçäo fótica intemitente aumentava a freqüência dos abalos mioclônicos que se tornavam bilaterais e sincronos, progredindo para uma crise tônico-clônica generalizada. Potenciais evocados e RMN em um caso foram normais . Drogas anticonvulsivantes foram ineficazes no controle das mioclonias. O diagníostico de encefalomiopatia mitocondrial foi realizado através do achado em espécimes musculares de membranas basais espessadas, com degeneraçäo miofibrilar e um número elevado de mitocondrias distribuídos perifericamente e com uma matriz densa, granular e com alguns vacúolos. Os achados clínicos e eletrográficos sugerem uma origem subcortical para esta síndrome mioclônica