Abstracts

A Novel Clinical Triad of Epileptic Visual Hallucinations, Nystagmus and Migrainous Headaches with Epileptogenic Zone in the Posterior Cerebral Quadrant. A Novel Case Series and Literature Review

Abstract number : 2.429
Submission category : 18. Case Studies
Year : 2018
Submission ID : 501137
Source : www.aesnet.org
Presentation date : 12/2/2018 4:04:48 PM
Published date : Nov 5, 2018, 18:00 PM

Authors :
Jaivir S. Rathore, Johns Hopkins University School of Medicine

Rationale: A novel series of three cases with complex semiology of visual hallucinations (VH) with nystagmus epilepticus (NE) and migrainous headaches (MH). Visual Hallucinations with Nystagmus Epilepticus (VHNE) is a rare phenomenon of transient, rapid and repetitive saccades associated with epileptic seizures. We present a unique cluster of VHNE without any motor manifestations; presenting with headaches ranging from mild constant headache to severe status migrainosus (MH). With less than 50 cases of nystagmus epilepticus published in literature, to the best of our knowledge, case series with such a novel complex semiology and clinical triad of VHNEMH has never been reported before. Methods: Three clinical cases of patients with age ranging from 27 to 51 year old right handed females with history of right MCA stroke in two of them and suspected focal cortical dysplasia in the  left posterior quadrant in the third patient. All these patients presented with visual hallucination, “Dancing eyes”, acute worsening of chronic intermittent to constant migrainous headaches. The patients reported the above clinical triad without oscillopsia, vertigo, alteration in speech, consciousness or motor symptoms. Results: Cluster of episodes with semiology of left or right conjugate eye deviation horizontal nystagmus with a rapid component (contra lateral to the ictal onset zone) and rightward slow phase without crossing midline. Video EEG showed seizure onset in the right (n=2) or left (n=1) posterior temporo-occipital region lasting from 30-110 seconds. Comprehensive workup was unremarkable. Migraine headache regimen provided partial relief. Visual hallucinations, nystagmus, headaches and EEG seizures totally resolved with antiepileptic medication and did not recur at 24 month follow-up in at least one of the cases and improved in the other two. Conclusions: The clinical triad of VHNEMH without any motor seizure or vertigo is a novel phenomenon. A few cases of NE due to ictal discharges from theposterior quadrant have been reported but none with the clinical triad of VHNEMH semiology. Video EEG must be considered in refractorymigrainous headaches or nystagmus to rule out ictal phenomenon. This can be best explained as epileptic activation of the cortical saccaderegions (including the frontal, supplementary and parietal eye fields causing a rapid phase of nystagmus contralateral to the ictal onsetzone and a slow ipsilateral phase. This novel case series not only has an epileptozenic zone localization value but also highlights how mood disorders and headaches are often misdiagnosed. Clinical video, EEG and MRI will be presented. Funding: None