Partial Epilepsy of Long Duration: Changing Semiology with Age

There are few data on changing patterns of localization-related epileptic syndromes with time and particularly on changes in seizure semiology as patients age. We retrospectively reviewed 53 patients aged >60 years who had had partial epileptic seizures for a mean duration of 44 years. In 20 patients, seizures became progressively less elaborate and briefer with time. In 29 patients, seizures were unchanged. In four patients, seizures worsened, with the appearance of drop attacks, secondarily generalized seizures, increasing drug resistance, and mental deterioration. These latter 4 patients had cerebrovascular disease with multiple strokes.     

Focal inhibitory seizures as the presenting sign of ischemic cerebrovascular disease

Recurrent episodes of transient neurological dysfunction occurring in a patient with evidence of recent multiple lacunar infarctions were at first diagnosed as transient ischemic attacks (TIAs), but later proved to be due to focal inhibitory seizures. The differential diagnosis between TIAs and partial epileptic seizures in patients with ischemic cerebrovascular disease may sometimes be difficult in the presence of uncommon clinical manifestations.

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Sommario Una paziente con quadro neuroradiologico di infarti lacunari multipli presentò come sintomatologia di esordio ricorrenti episodi di deficit neurologico transitorio che furono inizialmente interpretati come attacchi ischemici transitori (TIA), mentre la successiva osservazione dimostrò trattarsi di crisi epilettiche inibitorie somatiche. La diagnosi differenziale fra TIA e crisi epilettiche parziali nei pazienti affetti da malattia cerebrovascolare ischemica può presentare inattese difficoltà, in rapporto al possibile verificarsi di manifestazioni cliniche inusuali.

     

Differences between strychnine and penicillin epileptogenesis suggest a laminar organization of neocortical inhibition

Transient foci of epileptiform alteration in neuronal population activity were induced by microinjection of strychnine sulfate into different layers of cat striate cortex. Potentials evoked by visual field-specific photic stimulation were recorded from microelectrodes at the injection site and in adjacent laminae. Epileptogenesis, characterized by an enhancement of the normal primary response followed by the development of a large late potential, occurred only with strychnine injections into superficial pyramidal layers 2 and 3. By contrast, stellate layer 4 has been shown to be most susceptible to epileptogenic effects of penicillin and bicuculline. Since disinhibitory convulsants should be most effective where their actions antagonize the prevalent type of inhibition, these findings suggest that there may be a laminar segregation of neocortical inhibition, possibly glycine-mediated in layers 2–3 and probably gamma aminobutyric acid (GABA)-mediated in layer 4.     

Epileptiform burst responses in ventral vs dorsal hippocampal slices

Field potentials from area CA1 evoked by stimulation of the Schaffer collaterals were compared in dorsal and ventral hippocampal slices of rat brain. Responses were categorized into 5 response types on the basis of their morphology, ranging from simple (single spike component) to complex (multiple spike components). A higher percentage of ventral slices, compared to dorsal slices, responded with a complex morphology under normal and increased K+ concentrations. Thus, there was a significantly greater tendency for cells within the ventral hippocampus to generate burst responses.     

Ictal Behaviors During Nonepileptic Seizures Differ in Patients with Temporal Lobe Interictal Epileptiform EEG Activity and Patients Without Interictal Epileptiform EEG Abnormalities

Summary: Purpose: Ictal behaviors during psychogenic non-epileptic seizures (NES) vary considerably among individuals, and can closely resemble common semiologies of epileptic seizures (ES). We tested the hypothesis that behaviors during NES in patients who have temporal spikes would more closely resemble behaviors during ES in patients with temporal lobe epilepsy than would behaviors during NES in patients who do not have EEG spikes.
Methods: We identified 20 patients who had interictal temporal EEG spikes and EEG-video recorded NES (Study Group), 133 patients with temporal EEG spikes and recorded ES, without NES (Epileptic Group), and 24 patients with recorded NES and no epileptiform EEG abnormalities, without ES (Nonepileptic Group).
Results: The hypothesis was supported with regard to ictal motor behaviors. Motionless staring or complex automatisms occurred mainly during NES in the Study Group and during ES in the Epileptic Group. In contrast, convulsive movements or flaccid falls were most common during NES in the Nonepileptic Group. Duration of unresponsiveness was longer, and there were fewer postictal states in NES both in the Study and Non-epileptic Groups. Unresponsiveness was briefer and postictal states were more consistent in ES in the Epileptic Group, however.
Conclusions: Stereotyped motor activities during NES presumably represent learned behaviors. Processes underlying acquisition of ictal behaviors of NES probably differ in patients with interictal epileptiform EEG abnormalities compared to those without. Prior experiences and temporal lobe dysfunctions that are associated with epilepsy, and psychological characteristics that are unrelated to interictal epileptic dysfunctions, may determine ictal behaviors during NES.     

Clobazam Has Equivalent Efficacy to Carbamazepine and Phenytoin as Monotherapy for Childhood Epilepsy

Summary: Purpose: To compare the effectiveness of mono-therapy clobazam (CLB) to carbamazepine (CBZ) and phenytoin (PHT) in children with epilepsy.
Methods: Children aged 2–16 years with newly diagnosed epilepsy or previous failure of one drug (for poor efficacy or side effects) were assigned to one of two study arms and then randomized–CLB versus CBZ or CLB versus PHT. Eligible children had partial epilepsies or only generalized tonic-clonic seizures. After a drug initiation protocol, monotherapy treatment mimicked the usual routines used by Canadian child neurologists. Blinding used a "double dummy" technique with blinded medication serum levels (6–point scale). Intention to treat analysis using survival curves assessed the primary end-point–length of retention on the initial medication during the year after randomization.
Results: Fifteen centers entered 235 patients: 159 randomized to CLB versus CBZ and 76 to CLB versus PHT. Altogether, in all study arms, 119 received CLB, 78 CBZ, and 38 PHT. Overall, 56% continued to receive the original medication for l year with no difference between CLB and standard therapy (CBZ and PHT). Seizure control was equivalent for all three medications, as were side effects. PHT and CBZ induced more biologic side effects, such as rash, while CLB induced slightly more behavioral effects. Tolerance developed in 7.5% of patients receiving CLB, 4.2% with CBZ and 6.7% with PHT.
Conclusions: CLB should be considered as "first line" monotherapy along with CBZ and PHT for all partial and selected generalized childhood epilepsies.     

Self-perceived quality of life of Chinese elderly people in Hong Kong

Central to the philosophy of occupational therapy is a commitment to the improvement of clients' quality of life (QOL). There has been a growing interest in the QOL of elderly people in Hong Kong. Social and cultural differences may preclude the application of QOL findings for elderly people in Western countries to their Hong Kong counterparts. This study, which is the preliminary phase of a larger study, explored the QOL of Chinese elderly people in Hong Kong from their own perspective. Focus group interviews were conducted with six Chinese community-residing elderly people who identified the following domains and component elements as being important to their QOL: physical and functional well-being (good health, leisure), psychological well-being (life satisfaction, happiness), social well-being (social interaction, social network/support) and economic well-being (money, housing). Further investigation of factors including self-concept, self-pride, personal autonomy, role fulfilment, and coping ability is required to determine if these are QOL elements for this population. Participants' culturally related philosophical beliefs were found to influence their QOL. Copyright © 1998 Whurr Publishers Ltd.     

Ictal HMPAO-Single Photon Emission Computed Tomography Findings in Reading Epilepsy in a Japanese Boy

Summary: Reading epilepsy is rare. We report a 14-year-old right-handed Japanese boy who had had jaw jerking only while reading since age 12 years. The episodes occurred every time he read an English textbook and some-times during prolonged reading of a Japanese textbook. The jaw jerking evolved to generalized tonic-clonic seizures (GTCS) on only two occasions during prolonged reading aloud. Routine EEGs showed no abnormality. After a few minutes of reading, however, the EEG showed bilateral 2-Hz, 150-μ V spike-wave complexes with left frontotemporal accentuation, accompanied by jaw jerking. Ictal single photon emission computed tomography (SPECT) with [^99mTc]hexamethylpropylene amine oxime (HMPAO) showed focal hyperperfusion of the frontal lobes bilaterally and of the left temporal area. Interictal SPECT and magnetic resonance imaging (MRI) were normal. The combination of valproate (VPA) and clonazepam (CZP) almost eliminated his symptoms. Ictal SPECT is a useful technique for seizure localization in reading epilepsy.     

Vagus Nerve Stimulation for Symptomatic Generalized Epilepsy: A Pilot Study

Summary: Purpose: Patients with symptomatic generalized epilepsy (SGE) may have antiepileptic drug (AED)-resistant mixed generalized seizures. Vagus nerve stimulation (VNS) reduces partial seizures and may help SGE.
Methods: We added VNS to stable AED therapy in five SGE patients. Nine-month postoperative VNS treatment seizure rates were compared to a 1 -month preoperative baseline.
Results: All patients had mixed generalized seizures, EEG generalized slow spike-and-wave and behavioral abnormalities. Median number of previous AEDs taken was 6 (range 5–12). Median baseline seizure rate was 75honth (range 29–1 10). VNS produced a median seizure rate production of -41% (range -40%–85%). Adverse events reported in one patient each were: incisional infection, choking sensation and voice change; and coughing (noted by two patients). One patient discontinued VNS due to coughing.
Conclusions: We conclude that VNS may be useful add-on therapy for SGE. A larger, controlled, and blinded trial may be warranted.     

Tissue distribution and disease manifestations of the tRNALys A»G(8344) mitochondrial DNA mutation in a case of myoclonus epilepsy and ragged red fibres

This man with myoclonus epilepsy and ragged red fibres (MERRF) syndrome due to the tRNA^Lys A»G⁽⁸³⁴⁴⁾ mutation of mitochondrial DNA (mtDNA) died of bronchopneumonia at 18 years of age. He had progressive clinical symptoms from 6 months of age manifesting as ataxia, myoclonic seizures, and muscle weakness. A postmortem examination revealed 91–99% mutated mtDNA in all 32 examined tissue samples, including various organs and different brain regions. The brain appeared without macroscopic changes, but microscopic examination showed degeneration with loss of nerve cells and gliosis affecting the globus pallidus, substantia nigra, red nucleus, dentate nucleus, inferior olivary nucleus, cerebellar cortex, and the spinal cord. Skeletal muscle showed cytochrome c oxidase deficient muscle fibres with proliferation of mitochondria. In addition to pathological changes of muscle and brain there were few morphological changes that could be attributed to his mitochondrial disease. These data support the concept that in patients with the tRNA^Lys A»G⁽⁸³⁴⁴⁾ mutation who are manifesting disease there are high levels of mutated mtDNA in all tissues, but only some tissues and brain regions are vulnerable.