Diagnostic Yield of Routine Electroencephalography With Concurrent Video Recording in Detecting Interictal Epileptiform Discharges in Relation to Reasons for Request: A Prospective Study of 1,080 Video-Electroencephalograms.

PURPOSE: The aim of this study was to prospectively analyze the sensitivity and specificity of routine electroencephalography with concurrent video recording (vEEG) in relation to the reasons for requesting the test and to investigate when routine vEEG should be requested. METHODS: We prospectively analyzed 1,080 consecutive vEEGs performed between April 2015 and April 2016. The requests for vEEG were classified as requests with a low suspicion of epilepsy (syncope, confusion or delirium, suspicion of psychogenic nonepileptic seizures, and paroxysmal focal neurological deficit) or requests with a high suspicion of epilepsy (first clinical seizure, suspected status epilepticus, follow-up study of a patient with epilepsy, and acute symptomatic seizures). Predominant vEEG findings (ictal and interictal epileptiform activities, diffuse, or focal slowing and triphasic waves) were analyzed, and sensitivity and specificity [ZERO WIDTH SPACE][ZERO WIDTH SPACE]values calculated. RESULTS: The most common indication for vEEG was a follow-up study of patients with epilepsy (38%), followed by first clinical seizure (19.3%) and suspected status epilepticus (11%). The respective specificity and sensitivity values were 93% and 58% for 235 vEEGs performed in children/adolescents (≤18 years), 95% and 40% for 533 vEEGs performed in adults (>18 ≤ 65 years), and 93% and 39% for 312 vEEGs performed in older adults (>65 years). Twenty-four patients with false-positive paroxysms had a clinical diagnosis of confusional state or paroxysmal focal neurological deficit. Neurologists and neuropediatricians with experience in managing epilepsy had higher specificity values than general neurologists or physicians (P = 0.012). CONCLUSIONS: In our series, vEEG abnormalities were mainly observed in patients with clinical findings highly suggestive of epilepsy. In confusional states, and paroxysmal focal neurological deficit vEEG could be indicated.

Multiple Intracranial Arteriovenous Fistulas in Cowden Syndrome.

Cowden syndrome is a rare autosomal dominant disease. It is characterized by multiple noncancerous tumorlike growths called hamartomas, which typically are found in the skin, oral mucosa, thyroid, breast, and gastrointestinal tract. It carries with it a potential risk of malignant transformation, especially of the breast and thyroid. In 80% of the cases, the human tumor suppressor gene, phosphatase and tensin homolog (PTEN), is mutated in the germ line. We report a patient with Cowden syndrome who presented with generalized seizure and left anterior temporal hemorrhage and a nontraumatic subarachnoid hemorrhage due to multiple intracranial arteriovenous fistulas (AVFs). We discuss previous reports about vascular malformations in patients with Cowden syndrome and PTEN mutations. Importantly, we hypothesize that the production of multiple AVFs in our patient was associated with PTEN mutation.

Pilomotor seizures: an autonomic semiology of limbic encephalitis?

PURPOSE: Ictal piloerection is an infrequent seizure semiology that is commonly overlooked as an ictal epileptic manifestation. Piloerection is considered to be principally caused by temporal lobe activity although frontal and hypothalamic seizure origins have been reported. The described etiology has shown a wide variety of structural causes such as mesial temporal sclerosis, tumors, posttraumatic, cavernomas and cryptogenic epilepsies. METHODS: We retrospectively reviewed the incidence of ictal piloerection in the clinical records of patients who underwent video-EEG monitoring (VEEGM) between 2007 and 2013 in a multicenter cooperative study. All patients presented refractory epilepsies and were evaluated with a protocol that included brain MRI, neuropsychology and VEEGM. RESULTS: A total of 766 patients were evaluated in four tertiary centers in Spain. Five patients showed piloerection as principal seizure semiology (prevalence 0.65%). The mean age at seizure onset was 39.6 years and the average epilepsy duration was 5.2 years (range 2-14) before diagnosis. Four patients were additionally examined with FDG-PET and/or SPECT-SISCOM. All presented temporal lobe epilepsy (TLE), three right-sided and two left-sided. A typical unilateral hippocampal sclerosis was described in 3 cases. The etiology detected in all cases was limbic encephalitis. Three had LGI1, one anti-Hu, and another Ma2 antibodies. CONCLUSION: Our series describes a so far not well-recognized autoimmune association of pilomotor seizures to limbic encephalitis. This etiology should be ruled out through a comprehensive diagnostic work-up even in cases of long-lasting TLE with typical hippocampal atrophy on MRI.