Electroencephalographic findings in acute subdural hematoma.

The aim was to determine the electroencephalographic (or electroencephalogram [EEG]) findings predictive of functional outcome in a subset of patients with acute subdural hematoma (SDH) with epileptiform activity on their EEG. Twenty-four patients who underwent evacuation for acute or acute-on-chronic SDH and with epileptiform activity on EEG were identified retrospectively. Their EEGs were reviewed and the findings categorized along with clinical information, the preoperative computed tomography (CT) scan, and functional outcome. Twenty-one patients (87%) had epileptiform discharges on EEG; 13 of them (62%) had midline epileptiform discharges and 9 of them (43%) had periodic lateralized epileptiform discharges (PLEDs). Both types of epileptiform discharges were significantly associated with the degree of midline shift on neuroimaging (P = 0.01, P = 0.04, respectively). Poor early outcomes were associated with the presence of bilateral (P = 0.03), midline (P = 0.04), and bilateral independent multifocal discharges (P = 0.09) on EEG. The EEG findings in this group of patients were complex. Epileptiform discharges were common, and specific types were associated with midline shift on neuroimaging and poor functional outcome at hospital discharge. Improvement in follow-up EEG examinations over time was predictive of good long-term functional outcome.

Clinical characterization of a kindred with a novel 12-octapeptide repeat insertion in the prion protein gene.

OBJECTIVE: To report the clinical, electroencephalographic, and neuroradiologic findings in a kindred with a novel insertion in the prion protein gene, PRNP. DESIGN: Clinical description of a kindred. SETTING: Mayo Clinic Alzheimer Disease Research Center (Rochester, Minnesota). SUBJECTS: Two pathologically confirmed cases and their relatives. MAIN OUTCOME MEASURES: Clinical features, electroencephalographic patterns, magnetic resonance imaging abnormalities, genetic analyses, and neuropathologic features. RESULTS: The proband was a woman with clinical and neuroimaging features of atypical frontotemporal dementia and ataxia. Generalized tonic-clonic seizures developed later in the disease course, and electroencephalography revealed spike and wave discharges but no periodic sharp-wave complexes. Her affected sister and father also exhibited frontotemporal dementia-like features, and both experienced generalized tonic-clonic seizures and gait ataxia late in the disease course. Genetic analyses in the proband identified a novel defect in PRNP, with 1 mutated allele carrying a 288-base pair insertion consisting of 12 octapeptide repeats. Neuropathologic examination of the proband and her sister revealed prion protein-positive plaques and widespread tau-positive tangles. CONCLUSIONS: This kindred has a unique combination of clinical and neuropathologic features associated with the largest base pair insertion identified to date in PRNP and underscores the need to consider familial prion disease in the differential diagnosis of a familial frontotemporal dementia-like syndrome.

Electroencephalographic characteristics of patients infected with west nile virus.

SUMMARY: : The EEG is helpful in the evaluation of patients with altered mental status and can provide clues for the underlying cause of certain entities. There are few descriptions of the EEG findings in patients with West Nile virus (WNV) infection. We describe the clinical presentation as well as the electroencephalographic findings in five patients with WNV encephalitis. Review of the records of all cases of WNV infection seen at the Mayo Clinic from 1999 to 2003, in which an EEG had been performed. Five patients with WNV encephalitis, in whom an EEG was performed, were found. All the patients had altered mental status and the EEG showed moderate to severe degrees of generalized slowing. Three of the patients had triphasic waves on the EEG. The patients did not have any metabolic disorder or electrolyte abnormalities that could account for these findings. EEG findings in WNV are consistent with an encephalopathic pattern and show varying degrees of generalized slow wave abnormalities and in some cases triphasic waves.

EEG changes in a patient with steroid-responsive encephalopathy associated with antibodies to thyroperoxidase (SREAT, Hashimoto's encephalopathy).

A 56-year-old woman presented with an acute confusional state and moderate global aphasia. Thyroperoxidase antibody level was elevated (3,890 IU/mL) and SREAT was diagnosed. MRI findings were normal. Cerebrospinal fluid examination revealed only a mildly increased protein. The initial electroencephalogram EEG showed slowing and markedly decreased amplitude over the left hemisphere and left temporal sharp waves. An EEG performed after treatment with intravenous steroids showed a significant improvement of the background slowing, which correlated with clinical improvement. One week later, the patient had an episode of forced head-turning and fencing posture to the right. The EEG shortly afterward showed slowing and a decreased amplitude over the right hemisphere. Continuous EEG monitoring was performed at the time of steroid treatment. Again, there was a significant improvement of the EEG after this treatment that correlated with the clinical condition. SREAT is characterized by fluctuations in mental status and variable EEG findings. These patients often show an excellent clinical improvement to immunosuppressive therapy, including corticosteroids. This case report documents the dramatic clinical and EEG improvement with steroid therapy.

Pattern-sensitive epilepsy: electroclinical characteristics, natural history, and delineation of the epileptic syndrome.

PURPOSE: To elucidate the electroclinical features and long-term outcome of patients with pattern-sensitive epilepsy. METHODS: We reviewed the clinical and electroencephalographic (EEG) findings of 73 (43 female and 30 male) patients in whom pattern-sensitive epilepsy was diagnosed at Mayo Clinic (Rochester, Minnesota, U.S.A.) from 1950 through 1999. We contacted patients and their relatives by letter or telephone to obtain the latest seizure and quality-of-life outcomes. RESULTS: The median age at onset of seizures was 12.8 years (range, 0.6-32.9 years). Most patients had absence, myoclonic, or generalized tonic-clonic seizures. Interictal epileptiform discharges in the EEG were detected in 61 (83.6%) patients and were generalized in 54 (74%). Paroxysmal epileptiform discharges in the EEG elicited with standard patterns were all generalized in two thirds of patients but were restricted to the posterior head region in one-third. Eight (11%) patients did not exhibit photosensitivity. Television was the most common precipitant [30 patients (41%)]. Twenty-nine patients gave a clear history of one or more seizures precipitated while viewing environmental patterns such as window screens, garments, tablecloths, and ceiling tiles; the rest of the patients admitted that they preferred to avoid looking at patterned objects because these objects made them uncomfortable. The electroclinical features suggested juvenile myoclonic epilepsy in 14 patients, progressive myoclonus epilepsy in three, progressive familial cerebellar ataxia with myoclonus in two, and severe myoclonic epilepsy of infancy in one. During a median follow-up period of 15.7 years, 25 (45.5%) of 55 patients who were followed up for > or =5 years achieved complete seizure remission. The median age at remission was 24.4 years. The absence of progressive neurologic disease was correlated significantly with remission; a family history of seizures showed a trend in favor of remission. More than two thirds of the patients did not consider the seizures an impediment to their family life or to educational and occupational achievements. CONCLUSIONS: Although pattern sensitivity as a trait occurs in various epileptic syndromes, pattern-sensitive epilepsy is a readily distinguishable subtype of the visually provoked reflex epilepsies. In our opinion, the location and extent of the excitable region or regions within the visual cortex concerned with different attributes of visual function dictate susceptibility to a specific trigger (intermittent light, pattern, or color) or closely related multiple triggers and the resultant electroclinical phenomenon.

EEG changes with carotid sinus baroreflex during carotid endarterectomy.

We describe EEG changes in three patients in whom the carotid sinus baroreflex was precipitated during carotid endarterectomy. In all instances, the EEG showed diffuse attenuation and slowing of the baseline anesthetic pattern in association with bradyvcardia. This can help alert the surgeon to the changes occurring as a result of the cardioinhibitory carotid sinus baroreflex and guide appropriate timely intervention.

Clinical, magnetic resonance imaging, and electroencephalographic findings in paraneoplastic limbic encephalitis.

OBJECTIVE: To analyze clinical presentation of and paraclinical test abnormalities in patients with paraneoplastic limbic encephalitis (PLE). PATIENTS AND METHODS: We retrospectively reviewed 24 patients seen at the Mayo Clinic in Rochester, Minn, between 1985 and 2002 in whom PLE was suspected. Patients were identified on the basis of clinical history and presence of cancer. Data were reviewed from magnetic resonance imaging (MRI), cerebrospinal fluid (CSF) analysis, electroencephalography (EEG), and paraneoplastic serologic studies. RESULTS: Common manifestations were cognitive dysfunction (92%), seizures (58%), and psychiatric symptoms (50%); 13 patients had small cell lung carcinoma; 11 had other malignancies. Paraneoplastic neuronal autoantibodies were found in 14 (64%) of 22 patients tested. Electroencephalography showed focal or generalized slowing and/or epileptiform activity, maximal in the temporal regions, in all 22 patients tested. Magnetic resonance imaging revealed increased T2 signal involving one or both temporal lobes in 15 (83%) of 18 patients. Cerebrospinal fluid test results were abnormal in 18 (78%) of 23 patients tested. Clinical or radiographic evidence of extralimbic involvement was documented in 12 (55%) of 22 patients. No abnormality on EEG, MRI, or CSF analysis correlated with a specific cancer type or with a specific paraneoplastic autoantibody. CONCLUSIONS: In patients with suspected PLE, EEG is invaluable for confirming cerebral dysfunction. Magnetic resonance imaging can show unequivocal involvement of temporolimbic structures and helps exclude other diagnoses. When EEG and cranial MRI are both normal, PLE is unlikely. Comprehensive testing for paraneoplastic neuronal nuclear, cytoplasmic, and ion channel autoantibodies is an important part of the evaluation, but negative results do not rule out PLE.

Lateralized and focal clinical, EEG, and FLAIR MRI abnormalities in Creutzfeldt-Jakob disease.

OBJECTIVE: Creutzfeldt-Jakob disease (CJD) is a rapidly progressive fatal prion disorder with typical clinical findings of dementia, motor dysfunction, and myoclonus and characteristic electroencephalographic (EEG) findings of bilateral synchronous periodic sharp waves. Advances in neuroimaging capabilities with diffusion-weighted and fluid-attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI) techniques have shown promise in the non-invasive diagnosis of CJD. This series illustrates the correlation between the lateralized and focal clinical, EEG, and MRI FLAIR sequence abnormalities in 8 patients (7 men and one woman 55-73 years old) with CJD. METHODS: A case series of 8 patients, evaluated at Mayo Clinic, who had a history of rapidly progressive lateralized or focal neurologic dysfunction and laboratory findings consistent with CJD between 1996 and 1999 were identified. EEG, MRI of the head with FLAIR sequence, and cerebrospinal fluid studies were performed in all patients. RESULTS: Mean time to death from symptom onset was 4 months. Symptoms were lateralized to the left hemisphere in 5 patients and to the right hemisphere in two. One patient showed bilateral occipital lobe involvement. In all patients, the EEG showed lateralized or focal periodic sharp waves that colocalized with clinical cerebral dysfunction. FLAIR MRI images revealed increased signal in the cortical ribbon and deep gray matter corresponding to the lateralized clinical and EEG findings in 7 patients. The other patient had bilateral occipital increased signal on FLAIR MRI. CONCLUSIONS: CJD may present with lateralized or focal cortical syndromes with colocalizing EEG and MRI findings. With the appropriate clinical history and laboratory evaluation, the corresponding areas of increased signal on FLAIR MRI provide supportive evidence of the disease. SIGNIFICANCE: CJD can sometimes present with more focal or lateralized clinical findings, and the colocalizing EEG and MRI findings can help make or confirm the diagnosis of CJD.

Electroencephalographic findings in Kufs disease.

OBJECTIVE: To characterize the electroencephalographic (EEG) findings in patients with Kufs disease, the adult autosomal recessive form of neuronal ceroid-lipofuscinosis. METHODS: We reviewed the EEG findings in 5 patients with biopsy-proven Kufs disease from our institution and 14 case reports of Kufs disease in the literature. The criteria used for patient inclusion were clinical evidence of a progressive neurodegenerative disorder, biopsy-proven evidence of Kufs disease, and EEG recordings during the course of the illness. RESULTS: One patient had phenotype A with generalized atypical spike and slow wave complexes and marked photoparoxysmal responses, particularly at low flash frequencies. Three patients had phenotype B with generalized slowing. One patient in the miscellaneous category showed focal sharp and spike waves and quasi-periodic slow waves maximal over anterior regions of the head. Review of the literature identified 14 case reports that met the inclusion criteria. CONCLUSIONS: The inheritance, mechanism, and manifestations of Kufs disease are not well understood. EEG findings may guide clinicians toward a confirmatory pathological diagnosis and distinguish various phenotypes of this disorder. SIGNIFICANCE: The EEG may assist in the diagnosis of Kufs disease.

EEG findings in steroid-responsive encephalopathy associated with autoimmune thyroiditis.

OBJECTIVE: To analyze the electroencephalogram (EEG) findings of patients with steroid-responsive encephalopathy associated with autoimmune (Hashimoto) thyroiditis. METHODS: We reviewed 51 EEGs and the clinical records of 17 patients (5 men and 12 women, 27-84 years old). RESULTS: All patients had mild to severe generalized slowing on the EEG which corresponded to the clinical severity of the underlying encephalopathy. Other findings included triphasic waves, epileptiform abnormalities, photomyogenic response, and photoparoxysmal response. Follow-up EEGs of 13 patients showed slowing in 7 and a return to normal in 6. Myoclonic jerks were recorded during the EEG study of 8 patients but did not have an EEG correlate. The EEG and clinical condition improved after treatment with corticosteroids. When encephalopathy recurred, the EEG showed corresponding abnormalities. CONCLUSIONS: EEG findings in steroid-responsive encephalopathy associated with autoimmune thyroiditis consist mainly of slow wave abnormalities that reflect the degree of severity of the underlying encephalopathy. The EEG findings often paralleled the course of the disease, showing improvement with improvement in the clinical condition and worsening with recurrence of symptoms. SIGNIFICANCE: The EEG is helpful in evaluating and following patients with steroid-responsive encephalopathy associated with autoimmune thyroiditis in reflecting the degree of central nervous system (CNS) involvement, in determining whether their condition is better or worse, and in ruling out other causes of encephalopathy.