Raju Yerra

Clinical characteristics and outcome in patients with psychogenic nonepileptic seizures.

Objectives: To examine baseline clinical features of psychogenic nonepileptic seizures (PNES) in a large cohort and to investigate outcome over a period of up to 10 years. Studies investigating PNES have been limited by differences in diagnostic criteria, short follow-up periods, and the use of limited outcome measures. Method: Patients with PNES were identified, using strict diagnostic criteria. Baseline neurological, neuropsychiatric, and neuroimaging data were obtained from medical records. Long-term outcome was assessed with ratings of seizures, psychopathology, and quality of life in a subset of the patients. Results: Patients with PNES (n = 221) experienced long delays in diagnosis (&mgr;, 5.6 years; standard deviation, 7.7 years) and high rates (>60%) of prolonged treatment with antiepileptic drugs. Compared with previous studies, a relatively low proportion (8.1% to 17.9%, depending on diagnostic criteria) had comorbid epilepsy. An unexpected finding was that 22.6% of PNES patients had magnetic resonance imaging abnormalities. Patients assessed at follow-up (n = 61) exhibited poor long-term outcomes with ongoing PNES, high rates of psychopathology, low rates of specialist follow-up, poor quality of life, and poor overall levels of functioning. Conclusions: These results demonstrate the need for earlier diagnosis of PNES and comorbidities and highlight the need for diagnostic and therapeutic approaches that combine neurological and psychiatric perspectives. PNES = psychogenic nonepileptic seizures; VEM = video-electroencephalographic monitoring; EEG = electroencephalogram; AEDs = antiepileptic drugs; MRI = magnetic resonance imaging; ES = epileptic seizures; QOL = quality of life.

MRI-identified pathology in adults with new-onset seizures

Objective: To determine the frequency and nature of potentially epileptogenic lesions on MRI in adults with new-onset seizures. Methods: We prospectively studied a consecutive series of 993 patients (597 males [61%]; mean [SD] age: 42.2 [18.8] years, range 14.3–94.3 years) who presented to an adult First Seizure Clinic over a 10-year period. The MRI scans, performed clinically on 3- and 1.5-tesla scanners, were reviewed for their diagnostic yield, nature of abnormalities, and their association with abnormal electrical activity on EEG. Results: MRI scans were acquired in 764 patients (77%); potentially epileptogenic lesions were detected in 177 (23%). The frequency of potentially epileptogenic lesions was higher in patients who were diagnosed as having an epileptic seizure (28%) than in those with a nonepileptic event (8%) (p < 0.001), and highest in those who had focal-onset seizures (53%) (p < 0.001). The most common lesion type in patients with focal seizures was gliosis or encephalomalacia (49%). Other common lesion types were tumors (15%), cavernomas (9%), and mesial temporal sclerosis (9%). Abnormal MRI and EEG were concordant in 18% of patients, with EEG being normal in 55% of patients with epileptogenic lesions. Conclusions: MRI reveals potentially epileptogenic lesions in a minority of patients with a newly diagnosed seizure disorder. Lesions are most common in patients who have experienced focal seizures. The presence of a potentially epileptogenic MRI lesion did not influence the chance of having an abnormal EEG.

Utility and validity of a brief cognitive assessment tool in patients with epileptic and nonepileptic seizures.

OBJECTIVE Cognitive impairment is not uncommon in patients with epilepsy, and may relate to the underlying pathophysiology of epilepsy, the effects of seizures, or epilepsy treatment. Formal neuropsychological testing is not available in many centers, and few cognitive screening tools have been validated in an epilepsy population. We aimed to ascertain the reliability and validity of a multidimensional cognitive screening instrument, the Neuropsychiatry Unit Cognitive Assessment Tool (NUCOG), in a mixed epilepsy population. METHODS One hundred sixty-one of 177 consecutive patients admitted to a video telemetry unit were assessed with the NUCOG and classified with respect to seizure semiology, and a subset (n=33) were formally neuropsychologically assessed. RESULTS Scores did not differ between patients with epileptiform, those with nonepileptiform, and those with mixed EEGs on the NUCOG, nor between patients with focal and those with generalized epilepsies. Patients with a temporal lobe focus performed more poorly in general, and in memory specifically, than patients with an extratemporal focus. Scores on the NUCOG subscales Memory, Attention, and Executive Functioning correlated significantly with neuropsychological testing of these same domains, although patients were not impaired on measures of language or spatial function. CONCLUSION The NUCOG appears to correlate strongly with neuropsychological functioning in a number of key cognitive areas affected in patients with epilepsy, and appears to robustly detect memory impairment in patients with temporal lobe epilepsy.

Creutzfeldt–Jakob disease, cerebral amyloid angiopathy and Aβ-related angiitis with neuropsychiatric manifestations:

This case highlights the concept that Creutzfeldt–Jakob disease (CJD) has a diverse phenotype and suggests that the presence of one neurodegenerative process may influence the pathology of another (Paquet et al., 2008). A 70-year-old man, Mr F.T., presented to a neurologist with a 3-month history of unsteady gait and falls. Prior to this, he was active and fit. His past medical history was significant for hypertension. His father had a history of Parkinson’s disease. Neurological examination at the time of presentation yielded subtle hypomimia but no other signs of parkinsonism. Over the next 3 months, Mr F.T. experienced a rapid physical and cognitive decline and was admitted to hospital. He presented with a fluctuating conscious state characterised by hypervigilance and disorientation to time and place. He had evolving, poorly systematised delusions involving staff and the hospital. He could not follow two-stage commands or perform delayed recall. Cerebrospinal fluid was 14-3-3 protein positive, consistent with CJD. Magnetic resonance imaging (MRI) of his brain showed bilateral diffusion restriction affecting the caudate heads and putamen with involvement of the medial thalamus (Figure 1(a)). These are typical imaging appearances of CJD. There were bilateral subcortical white matter changes (Figure 1(b)), reflecting chronic small vessel ischaemia. Widespread microhaemorrhages were present peripherally within the cortex, particularly in the temporal lobes and right parieto-occipital regions (Figure 1(c) and (d)), consistent with cerebral amyloid angiopathy (CAA). On post-mortem, neuropathological examination, spongiform encephalopathy was present throughout the cerebral cortex, basal ganglia and brainstem. There was cortical amyloid angiopathy (Figure 2(a)) with focal evidence for a granulomatous vasculitic response consistent with Aβ-related angiitis (ABRA). Beta-amyloid was colocalised in the vessel walls, and there was amyloid plaque in the cerebral cortex. There was a macroscopic occipital lobe haemorrhage associated with prominent amyloid angiopathy and extensive prion plaque in areas of overlying cortex (Figure 2(b)). The coexistence of CAA, ABRA and CJD is not often described. This case also adds to a small number of reports describing CJD associated with CAA (Gray et al., 1994; Keohane et al.,

Complex visual hallucinations and occipital seizures.

Background: We describe the presentation of a young woman with long-standing complex partial seizures with occasional secondary generalization, who presented with complex visual hallucinations (CVHs) and delusions. Methods: Routine biological workup including magnetic resonance imaging revealed an area of significant left-sided occipital gliosis. Video telemetry monitoring revealed a left occipital focus for the origin of the electrographic seizure discharge. Conclusion: CVHs occur in a range of organic states, including epilepsy, and can be understood in terms of the underpinning neuroanatomy and neurotransmitter systems of the visual system.