Rotational vestibular epilepsy from the temporo-parieto-occipital junction

Abstract

Transient signs and symptoms of CNS involvement have been reported in Charcot-Marie-Tooth disease type 1X (CMT1X). 1,2 We describe a patient with CMT1X and chronic progressive-relapsing inflammatory demyelinating CNS disease.Case report. A 44-year-old man was admitted because of subacute onset of gait ataxia, dysphagia, and dysarthria. He had experienced worsening stepping gait for the previous decade, and an EMG examination a year and a half before presentation had shown a demyelinating sensorimotor neuropathy. Otherwise, his medical history was unremarkable. Neurologic examination showed a wide based gait with ataxia worsening after eye closure, upper limb incoordination, and gaze-evoked nystagmus. There was weakness of foot dorsiflexion and toe extension. Tendon reflexes were absent. The patient was dysarthric and had dysphagia for liquids. EMG showed a mixed axonal-demyelinating polyneuropathy (table E-1 on the Neurology Web site at www.neurology. org). Visual evoked potentials (VEPs) were normal, whereas brainstem auditory potentials (BAEPs) showed alterations bilaterally (table E-1). Brain MRI showed multiple T2-hyperintense lesions in the pons, paraventricular region, and corpus callosum (see figure, A through C), without gadolinium (Gd) enhancement. Analysis of CSF showed increased protein content (82 mg/dL) with normal cell count. Oligoclonal IgG bands restricted to CSF were also detected. Sural nerve biopsy showed onion bulbs and loss of myelinated fibers, without inflammatory infiltrates. The patient was treated with IV methylprednisolone 1 g/day for 5 days, without appreciable benefit. One month later, during rehabilitation, he suddenly developed bilateral loss of visual acuity. VEPs showed marked prolongation of P100 wave latency (table E-1). He was treated again with IV methylprednisolone 1 g/day for 5 days, without benefit. Three years later, he was again admitted to our hospital. The neurologic examination showed severe gait ataxia which confined the patient to a wheelchair, and severe incoordination of upper limbs with marked action tremor. Dysarthria and dysphagia were moderate. Muscle strength was normal except for moderate weakness of tibialis anterior, extensor hallucis, and dorsal interossei. Urinary retention was also present. Brain MRI scans demonstrated increased T2-lesion load and one Gdenhancing lesion in the pons (figure, D). BAEPs and VEPs were unchanged. Treatment with IV immunoglobulins (0.4 g/kg/day) was withdrawn after 2 days because of hematuria. DNA analysis showed the mutation c490 CϾT, R164W in the GJB1 gene. The family history was reassessed. A brother showed stepping gait with bilateral severe deficit of ankle dorsiflexion and, to a lesser degree, plantar flexion. EMG showed a mixed sensorimotor polyneuropathy with conduction velocities in the same range of the index case. BAEP and VEP were unremarkable. Brain MRI was normal. Genetic analysis showed the same mutation of GJB1 gene identified in the index case.Discussion. Our index patient showed a CNS demyelinating disease th...

Seizure semiology in temporal lobe vs. temporal plus epilepsy using intracranial EEG monitoring

“…14 In this study, visual auras can differentiate TPE from TLE cases. Vestibular auras were reported by one patient in our sample who had TPE involving the temporooccipital junction, which is in line with the literature 8 and a reported case 15 that was later confirmed by cortical stimulation of the precuneus of the non-dominant parietal lobe to elicit rotatory vertigo. 16 Ictal motor signs, such as manual automatism and dystonic posture, were commonly observed in TLE and TPE.…”

Clinical and electrographic findings in epileptic vertigo and dizziness

“…Less frequently, Kahane et al found vestibular sensations by stimulating within the occipital, frontal, and insular cortex. Congruent but somewhat more expansive than these experimentally derived results, epileptic discharges on ictal EEG in symptomatic EVD were individually localized to frontal, 2 frontotemporal, 10 temporal, 11,12 temporo-parieto-occipital, 13 and parietal 14 areas, suggesting either processing of vestibular-related input across large cortical regions 15 or spread of excitation to or from nearby regions. Lesion studies in humans provide additional insights into the extent of cortical network processing of vestibular/graviceptive inputs.…”

Incidence of rotational vertigo in supratentorial stroke: A prospective analysis of 112 consecutive patients

“…We postulate that such sensations may represent seizure activity secondary to the ischemic event. Rotational and other vestibular sensations are a well recognized ictal phenomenon [11,[23][24][25]. Epileptiform discharges arising from areas covered from opercular tissue, such as the insular cortex, might not be easily detectable from scalp EEG-recordings, and one may assume that this was one of the reasons why previous reports may have failed to depict paroxysmal activities.…”