Ictal FDGPET and SPECT in hemifacial seizures due to cerebellar epilepsy-Case report.

The role of cerebellum in seizure generation is debatable. Semiology and electroencephalography (EEG) findings are non-specific and sometimes misleading, posing further difficulty in proving the epileptogenicity in pre-surgical workup. We report two cases of cerebellar lesions who presented with hemifacial seizures since the neonatal period and were refractory to antiepileptic drugs (AEDs). Both inter-ictal and ictal EEGs were non-contributory. Magnetic resonance imaging (MRI) showed a lesion in the cerebellum, in proximity to cerebellar peduncle in both the patients. (18) F-fluorodeoxyglucose-positron emission tomography (FDG-PET) and ictal single photon emission computed tomography (SPECT) showed focal hypermetabolism and hyperperfusion respectively, corresponding to the lesion on MRI in both the cases. Intraoperative electrocorticography showed rhythmic spikes confirming the epileptogenic nature of the lesion. Both patients were operated with a favorable surgical outcome. Histopathology was suggestive of a ganglioglioma in one child and a low-grade glioma in the other. Both cases illustrate that FDG-PET and SPECT can act as surrogate markers for invasive recordings to prove the epileptogenicity of cerebellar lesions, especially in resource limited settings.

Epilepsy surgery in children.

Approximately 60% of all patients with epilepsy suffer from focal epilepsy syndromes. In approximately 15% of these patients, the seizures are not adequately controlled with anticonvulsive drugs, and such patients are potential candidates for surgical treatment and majority are children. Epilepsy surgery in children, who have been carefully chosen, can result in either seizure freedom or a marked (>90%) reduction in seizures in approximately two-third of children with intractable seizures. In the multimodality presurgical evaluation approach, sufficient concordance should be established among various independent investigations, thus identifying the location and extent of the epileptogenic zone with a high degree of confidence. Early surgery improves the quality of life and cognitive and developmental outcome of the child. Surgically remediable epilepsies in children should be identified early and include temporal lobe epilepsy with focal lesions, lesional extratemporal epilepsies, hemispherical epilepsies, and gelastic epilepsy with hypothalamic hamartoma, and can be treated by resective or disconnection surgery. Palliative procedures include corpus callosotomy and vagal nerve stimulation for children with diffuse and multifocal epilepsies, who are not candidates for resective surgery. Deep brain stimulation in patients with epilepsy is still under evaluation. For children with "surgically remedial epilepsy," surgery should be offered as a procedure of choice rather than as a treatment of last resort.

Impact of JME on quality of life: Does a well-controlled epilepsy also hamper the psychological wellbeing?

PURPOSE: Epilepsy is a stigmatizing disorder and its diagnosis can have important psychosocial consequences on individuals, severely impacting their quality of life(QOL). There are numerous studies which have seen an adverse impact on the psychosocial aspects of life in patients with intractable epilepsy. The aim of this study was to assess the QOL in adult and adolescent patients with JME, which is largely a well-controlled form of epilepsy. METHODOLOGY: This was a hospital based cross-sectional observational study comprising of 50 JME patients. QOLIE-31-P and QOLIE-AD-48 questionnaires were used to assess QOL in adults & adolescents(11-17 years) respectively. The Mini international neuropsychiatric interview-version 7.0.2 and Brief psychiatric rating scale were used for screening of underlying psychopathology and if, the screening tests were positive then they were further evaluated and classified using DSM V and ICD 10. RESULTS: The mean QOLIE-31-P score was 64.65 ± 15.74. Majority of the adult patients had fair QOL (poor, fair and good QOL scores in 18 %, 54 % and 28 % respectively). Subscale scores in poor category were for the medication effects and pertaining to seizure worry.Among adolescent patients, the mean QOLIE 48 AD score was 69.15 ± 13.13. 50 % had fair QOL. Amongst those with poor QOL, majority of poor scores were for the attitude towards epilepsy. The QOL scores were significantly poorer in patients with uncontrolled seizures. 78 % of the patients had comorbid anxiety and depression, however syndromic psychiatric diagnosis was seen in 10.25 % and 2.56 % for anxiety and depression respectively. Presence of psychiatric symptoms did not influence QOL scores. CONCLUSION: QOL, in well controlled JME, is fair in majority of patients. QOL might improve if seizure worry is addressed and patients are educated about medication effects at the time of initial diagnosis. Vast majority of patients may experience minor psychiatric issues, which do need addressal for formulating a holistic and individualized treatment plan.

Lower limb monochorea from a globus pallidus infarct.

Movement disorders are common following cerebrovascular accidents and they can be hyperkinetic, including hemichorea and hemiballismus, or hypokinetic, as seen in parkinsonian disorders. Monochorea has also been reported due to stroke, albeit rarely. We report a 47-year-old gentleman who presented with a history of sudden onset choreiform movement of his left lower limb. On clinical examination his motor power was normal and there were no abnormal movements in any other limb. MRI of his brain was suggestive of an infarct in the right globus pallidus interna extending up to the posterior limb of the internal capsule. He was treated with clonazepam and trihexyphenidyl. His movements improved significantly within 3 months. Monochorea in a lower limb due to an infarct in the globus pallidus interna is unusual and highlights the complexity of the pathophysiology of chorea.

Babinski the great: Failure did not deter him.

Joseph Babinski (1857-1932) was born on November 17, 1857. He worked in a clinical arena dominated by Charcot and a focus on hysteria. His primary aim was in trying to find the reliable clinical signs to distinguish organic from non-organic disease of the nervous system. He was considered as masterly diagnostician, relying considerably less on neuropathological reports. Babinski's first attention to the reflex of the toes occurred during a chance observation of the contrasting responses between two female patients, one a hysteric and the other a hemiplegic. He first published description of his famous "sign" in 1896. Babinski's love for research works could be gauzed from his desire to publish and by the age of 27 years, he had to this credit, 12 important articles, mainly concerned with histological and neurological themes and one of his articles on the basic description of muscle spindles was considered to be a significant one. He was awarded the doctorate degree in 1885. Babinski introduced the concept of pithiatism, meaning "curable by suggestion." He anticipated the emergence of neurosurgery in France and only 6 days prior to his death he is on record to have said that his most vital contribution to the cause of neurosciences was not the sign he described, but that he could goad Clovis Vincent and Martel to take up neurosurgery as a specialty.