Weight change associated with valproate and lamotrigine monotherapy in patients with epilepsy.
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Biomedical subjects
Publications and source records attributed to E Trinka.
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OBJECTIVE: To analyze the clinical features and family history of patients with idiopathic generalized epilepsy (IGE), with pure grand mal (GM), divided into epilepsies with GM occurring exclusively on awakening (GMA) and random GM (RGM). METHODS: We studied retrospectively 98 patients from a large epilepsy outpatient clinic. All patients had a full clinical examination and computed cerebral tomography scans (CCT) or magnetic resonance imaging (MRI) when feasible. We analyzed seizure type, seizure frequency, provocative factors, prognosis, electroencephalography (EEG) findings and family history. RESULTS: Sixty-eight patients had GMA and 30 had RGM. The mean age at seizure onset was 16.6 years (+/-6.3 S.D., range: 5-41) and 16.7 years in those with RGM (+/-7.5 S.D., range: 4-42, NSD). Patients with GMA had a longer course of active epilepsy (median 8.5 years) compared to RGM (median 2 years). Seizure-provoking factors, especially sleep deprivation, were significantly (P=0.001) more common in patients with GMA (52/68, 77%) than in the group with RGM (13/30, 43%). Of all patients, 23% (23/98) reported first degree relatives with seizures or epilepsy. Pure GM was found in 41% (12/29) of affected first degree relatives, other idiopathic generalized epilepsy syndromes were less frequently observed (4/29, 14%). The concordance rate was high within the syndrome - none of the patients with RGM had an affected relative with GMA and vice versa only two of affected relatives of GMA patients had RGM. CONCLUSION: GMA seems to be associated with a longer duration of active epilepsy, a higher relapse rate and a stronger tendency to be precipitated by seizure provoking factors. The different concordance rates between the syndromes suggest a genetically different background.
BACKGROUND: Several studies demonstrate that the P3 component of the event-related potentials (ERP) is generated by mesial temporal structures. The P3 is considered as endogenous information processing component independent from modality. PURPOSE: The aim of this investigation was to study whether the P3 latency elicited by auditory and visual stimuli under different attentional states is influenced by the mesial temporal lobe epilepsy (mTLE). METHODS: The event related P3 component of 10 right handed patients (7 m/3 f, mean age 31.3 years (SD 7.3), median 31.5, range 20-47) with unilateral mTLE were studied and compared with 10 age matched controls (5 m/5 f, mean age 36.6 years (SD 12.6), median 32.1, range 22-48). Diagnosis of mTLE was based on seizure description, clinical history and examination, prolonged video-EEG monitoring, magnetic resonance imaging (MRI), interictal and ictal SPECT. P3 potentials were elicited with a visual and auditory three stimulus discrimination paradigm. RESULTS: The latency of the P3 response to the auditory stimulus under automatic and effortful attention was delayed (423.3 ms (SD 60.4) and 436.9 ms (SD 83.4)) compared with a control group (359 ms (SD 42.4) and 354.2 ms (SD 39.7); P = 0.039 and P = 0.012, ANOVA), while the P3 latencies to visual stimuli under both tested attentional states (407.3 ms (SD 52.9) and 390.6 ms (SD 40.2)) did not differ from controls (378.2 ms (SD 48.6) and 378.2 ms (SD 55.4)). P3 latencies to auditory novel paradigm in patients with right sided mTLE, but not left sided mTLE, differed significantly from controls (P = 0.01). CONCLUSION: The results suggest a modality dependence of the P3 component in patients with mTLE. The novelty processing of auditory stimuli is disturbed in patients with right sided mTLE.
We report a patient with early infantile epileptic encephalopathy (EIEE) with suppression-burst (Ohtahara syndrome) associated with olivary-dentate dysplasia and agenesis of mamillary bodies is reported. Although those with Ohtahara syndrome are a heterogeneous group, virtually all reported cases are secondary to neuronal migrational disorders, sometimes only identified by detailed neuropathologic examination, as in this case report, which describes mamillary body agenesis as a not-yet-recognized anomaly associated with Ohtahara syndrome. All children with Ohtahara syndrome should have high-resolution magnetic resonance imaging (MRI) and detailed postmortem neuropathologic examinations.
We report a 52-year-old woman who underwent otoneurosurgery to resect acoustic neurinoma. Bone reconstruction was performed with an aluminium (Al)-containing cement. Six weeks later the patient suffered from loss of consciousness, myoclonic jerks, and persistent grand mal seizures, clinical symptoms that resembled those of lethal dialysis encephalopathy of the 1960s and 1970s. She died 6 months later because of septic complications. Light- and electron-microscopic investigation of the central nervous system (CNS) showed pathognomonic Al-containing intracytoplasmic argyrophilic inclusions in choroid plexus epithelia, neurons, and cortical glia. These changes are characteristics of dialysis-associated encephalopathy (DAE), induced nowadays by long-term ingestion of Al-containing drugs (and with benign clinical courses). Atomic absorption spectrometry showed an increase of mean bulk Al concentration of the cortex and subcortex up to 9.3 microg/g (normal range <2 microg/g); laser microprobe showed the increase of Al in subcellular structures. This unique case again shows the extraordinary neurotoxicity of Al, which was, in our patient, initiated by an amount of about 30 mg Al and apparently caused by direct Al access to the brain parenchyma via a cerebrospinal fluid leakage.
OBJECTIVE: To study the prognosis of patients with complex partial seizures (CPS) with or without simple partial (SPS) and secondarily generalized tonic-clonic seizures (GTCS) and to analyze the factors related to the degree of medical responsiveness. MATERIAL AND METHODS: A total of 266 adult patients with CPS were included in a hospital based observational survey with a follow-up of 2 to 25 years. Clinical characteristics, seizure frequency, electroencephalography (EEG), cerebral computed tomography (CCT) and magnetic resonance imaging (MRI) findings were analyzed. Patients were categorized according to their degree of medical responsiveness into one of three groups: seizure free, improved control (>50% seizure reduction) and poor control. RESULTS: Mean age at follow-up was 44.7 years (SD 14.7, range 19-93). Mean age at seizure onset was 18.1 years (SD 14.7, median 15, range 1-79). Complete seizure control was achieved in 40%, improved seizure control in 36% and poor seizure control in 24%. Patients entered remission after a mean period of 15.7 years (SD 12.6, median 13, range 1-54) of active epilepsy. A third of all seizure-free patients were still in remission 6.1 years (SD 5.3, median 3.5, range 1-18) after discontinuation of antiepileptic drugs (AED). Patients with poor seizure control had a significantly younger age at onset (P<0.01), a higher initial seizure frequency (more than 3 per month) (P<0.01), abnormal neurological examination (P<0.01), and were more often mentally handicapped (P<0.01). Multiple logistic regression analysis revealed a high initial seizure frequency, mental handicap and an abnormal neurological examination as independent risk factors for poor seizure control. A positive family history, a history of febrile convulsions and/or psychosis, an abnormal EEG or MRI was not predictive of poor outcome. CONCLUSIONS: Not all patients with CPS were medically intractable. Seizure remission can be achieved after a long time of active epilepsy. Poor seizure control was associated with a high initial seizure frequency, mental handicap and abnormal neurological examination.
The authors describe six patients with medically refractory temporal lobe epilepsy whose seizures were characterized by an aura of ictal urinary urge. All seizures originated in the nondominant temporal lobe as evidenced from interictal spikes, ictal EEG, and MRI. Ictal SPECT, which was obtained in two patients, showed a hyperperfusion of the insular cortex, indicating a critical role of the insula for the generation of this symptom. Ictal urinary urge represents a new lateralizing sign indicating a seizure onset in the nondominant temporal lobe.
The authors studied prognostic factors for surgical treatment in 22 patients with intractable postencephalitic epilepsy. A small subgroup of patients (9/22) had a positive outcome after resective surgery. They had a higher functional level after encephalitis as measured by Glasgow Outcome Score, a longer time interval between encephalitis and onset of seizures, and localization of ictal EEG to one temporal lobe. The other patients had devastating seizures with poor outcome after surgery.
The second lumbrical-interosseus distal motor latency (2LI-DML) was compared prospectively in 450 hands. Median nerve function was assessed by standard motor and sensory electrophysiological tests. In a control group of 100 hands the upper limit of normal for the 2LI-DML was 0.5 ms. In all hands studied the correlation coefficients of 2LI-DML were higher with sensory nerve tests than with motor studies. Carpal tunnel syndrome (CTS) was diagnosed clinically in 276 hands, and 174 showed no clinical signs of CTS. The 2LI-DML was prolonged in 269 of the 276 hands, with clinical signs of CTS and normal in 170 of 174 non-CTS hands. Thus the 2LI-DML resulted in a sensitivity of 97.5%. On the other hand, combining the standard tests yielded a sensitivity of 98.5 %. In 31 of 36 additional hands a lumbrical response was recorded, although motor and sensory responses form standard median nerve conduction studies were absent, and the 2LI-DML was substantially prolonged. The 2LI-DML therefore represents a highly sensitive, fast, easy-to-perform, and cost-efficient method to study median nerve function across the wrist and may help to localize the lesion in cases in which standard electrophysiological methods fail.
UNLABELLED: Idiopathic Parkinson's disease (PD) is the most common neurodegenerative disorder. An important step in diagnosing the disease has been achieved with the development of the cocaine derivative [123I] beta-CIT for single photon emission computed tomography (SPECT). The aim of this study was to demonstrate the disease progression by repeated measuring presynaptic dopamine transporter density and relating it to clinical data. METHODS: The presynaptic dopamine transporter density of 15 PD patients was measured two times with a mean interval of 15 months. All patients were clinically assessed at the time of the experiments according to the classification scheme of Hoehn and Yahr. 11 healthy volunteers were used as a control group. [123I] [-CIT was injected intravenously and measured with a triple-headed camera twenty hours later. The pictures were evaluated semiquantitatively by using the ratio of specific to non-displaceable binding. RESULTS: Presynaptic dopamine transporter density differed significantly between controls and PD patients. A significant correlation between imaging data and clinical stages (H/Y I -27%, H/Y II -40%, H/Y III -58%) was observed for the patient group in the initial experiment. The subsequent decrease of dopamine transporter binding depended on the initial clinical stage (H/Y I -6.81%; H/Y II -6.05%; H/Y III -1.25%) of the patients, and regression analysis revealed that. 91.4% of the variance of the second measurement were predicted by the initial measurement. No correlations were found for age, gender and disease progression. All patients were treated with L-DOPA and those given a higher dose showed a more rapid decrease of dopamine transporter density. This result could be interpreted as an indication for in vivo neurotoxicity of high concentrations of L-DOPA. CONCLUSION: We conclude that combining [123I] beta-CIT with SPECT imaging is not only a powerful tool for diagnosing PD patients, but may also be used to demonstrate neurodegeneration in vivo.
The putative generators of the event-related P3 component are still a matter of debate. There is reasonable evidence that the mesial temporal lobe structures are crucial in the generation of event-related potentials. Transient global amnesia (TGA) is characterized by anterograde and retrograde amnesia without neurological deficits in which a temporal hypoperfusion is the most likely pathomechanism. The aim of this study is to evaluate a possible delay in the P3 latency in a cohort of patients with TGA (n = 18). We used classic oddball paradigms with visual and auditory stimuli. The P3 latencies were compared to 18 age-matched patients with transient ischaemic attacks (TIAs) in the carotid artery territory and 23 age-matched controls. The mean age of the patients was 60.1 years (SD 14.1), in the TIA group 64.7 (SD 12.1) and 57.7 (SD 10.1) in the controls; the mean time interval between onset of TGA symptoms and performance of P3 investigations was 4.3 days (range 1-7 days); and the mean latencies measured at the vertex (Cz electrode) were 422.9 ms (SD 44.6) for the TGA patients, 436.4 ms (SD 62.1) for the TIA group, and 409.3 ms (SD 48.5) for the controls in the visual paradigm and 371.7 ms (SD 41.7), 399.7 ms (SD 51.2) and 385.3 ms (SD 46.5) in the auditory paradigm for the TGA, the TIA and the control groups. The P-values were not significant. These results suggest different neuronal networks in the generation of scalp P3 from those responsible for the disturbance of episodic memory in TGA.
The P3 potential is accepted as a neurophysiological correlate of memory and attention. Delayed latencies were reported in different forms of dementias. Although the generator sites are still under debate, the thalamus may play a crucial role. The aim of this study was to investigate the influence of an unilateral thalamic ischaemic infarction on P3 generation. The event-related P3 component of six patients (2 male, four female; mean age 47 years, range 22-63 years) with unilateral thalamic ischaemic infarction was studied and compared to age-matched controls (five male, nine female; mean age 45.8 years; range 22-69 years). All patients underwent full clinical examination, CCT, and MRI scan. P3 potentials were recorded with a visual three stimulus discrimination paradigm. The mean P3 latency of the patient group to the target stimulus was delayed (469.7 ms, SD = 36.8) compared with the controls (378.8 ms, SD = 51.5; P < 0. 05). The mean P3 latency to the unexpected stimulus was delayed in patients with thalamic infarction compared with controls [477 ms (SD = 46.6) vs. 381.2 ms (SD = 48.5); P < 0.001). Delayed P3 components of the event-related potential (ERP) were recorded in six patients with unilateral thalamic infarction, suggesting an important role of the thalamus in the generation of the P3 potential.
We report two adults who received gabapentin (GBP) and subsequently developed behavioural side effects. Indications for GBP treatment were newly diagnosed epilepsy in one and painful paraesthesiae in the other. Both had no past history of psychiatric or behavioural disorder. Abnormal behaviour consisted of elevated mood, euphoria, and increased energy in both patients, and pressure of speech and decreased need for sleep in one of them. These symptoms were transient and fully reversible. One patient had to discontinue GBP. Behavioural changes were not related to seizure activity. They should be recognized as a possible side effect of GBP treatment in adults.
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The largest group of neurodegenerative disorders are extrapyramidal diseases, especially parkinsonism. The development of the cocaine derivative [123I] beta-CIT and single photon emission tomography (SPET) may help in the diagnosis of these patients. The aim of this study was to demonstrate the diagnostic value of this method and its relationship with clinical data. Ninety-eight individuals were investigated: 11 healthy volunteers, 58 patients with idiopathic Parkinson's disease (IPD) and 29 patients with symptomatic parkinsonism (SPD). All patients with parkinsonism were staged according to the clinical classification of Hoehn and Yahr. [123I] beta-CIT was injected intravenously and a triple-headed camera was used to obtain images 20 h later. The images were evaluated visually and semi-quantitatively to obtain comparable values (ratio: specific to non-displaceable binding). The ratios differed significantly between controls and IPD patients. A significant correlation also existed between the ratios and clinical stages. In 11 hemiparkinsonian patients, a significantly diminished ratio was demonstrated not only contralateral to the affected side, but also in the clinically silent striatum. A clinical threshold at a reduction of 34% [123I] beta-CIT binding was calculated in this group. The ratios of all SPD patients in our study did not differ significantly from those of the healthy volunteers. According to the clinical degree of symptoms, the more severe subgroup showed a diminished mean ratio of 22% and therefore could not be clearly differentiated from mild IPD. In contrast, ratios were significantly different when comparing groups of the same clinical severity. We conclude that this method is not only a powerful diagnostic tool in IPD patients, but it is also possible to differentiate between IPD and SPD patients, if clinical aspects are also included.
OBJECTIVES: The aim of this study is to characterize the clinical features and prognostic factors for intractable, post-encephalitic epilepsy. METHODS: We studied retrospectively 42 patients (26 men) evaluated between 1982 and 1999. MRI, neuropsychological findings, interictal and ictal scalp EEG were reviewed for all patients. Fifteen patients had additional stereo EEG (SEEG) studies. RESULTS: The mean age at encephalitis was 17 years (SD = 15.5); etiology was identified in 18 patients. During the acute illness, 79% had status epilepticus (SE) or recurrent seizures and 76% were in coma; mean Glasgow outcome scale (GOS) was 3.6 (SD = 0.8). The mean latency to seizure onset was 0.8 years (SD = 1.9). The majority (72%) presented with complex partial seizures with or without secondary generalization. According to interictal epileptiform findings and the predominant seizure onset pattern as found on scalp EEG, patients were unilateral temporal (UTLE) = 8, bilateral temporal (BTLE) = 12, and extratemporal/multifocal or generalized (ETMFE) = 22 patients. MRI atrophy and/or signal changes were unilateral temporal in 7 (18%), bilateral temporal in 5 (13%), multilobar/diffuse in 20 (51%), and absent in 7 (18%). ANOVA revealed significant differences in mean GOS between UTLE versus BTLE and ETMFE (4.7 versus 3.2 versus 3.6; p < 0.0001), but not in age at encephalitis. Latency to the first unprovoked seizure was shorter in patients with ETMFE compared to UTLE and BTLE (p < 0.01). Surgery was performed in 24 patients. The best outcome was obtained in UTLE (7/8 class I and II). In the others, outcome was poor in the majority (13/16 class III and IV). CONCLUSION: There is a small subgroup of patients with postencephalitic UTLE for whom the outcome is favorable. The majority of our patients had multifocal seizure onset with BTLE and ETMFE, and poor outcome after surgical treatment.
A case of complex partial status epilepticus (CPSE) with high dose treatment of tiagabine (TGB) is reported. Seizure aggravation and CPSE developed after stepwise increase of TGB to a dose of 60 mg per day as add-on treatment to carbamazepine (CBZ) 1200 mg/day and vigabatrine (VGB) 1000 mg/day. The EEG during CPSE showed bilateral rhythmic slow activity. Clinical symptoms of CPSE and the EEG normalized after i.v. treatment with clonazepam. The literature and the possible mechanism of this paradoxical phenomenon are discussed.