Neurology India Medknow Publications on behalf of the Neurological Society of India ISSN: 0028-3886 EISSN: 1998-4022 Vol. 58, Num. 2, 2010, pp. 326-327

Neurology India, Vol. 58, No. 2, March-April, 2010, pp. 326-327

Letter To Editor

Reversible photoparoxysmal response at 1 Hz in a patient with posterior fossa mass lesion

Gopal Krishna Dash, Mahesh Pundlik Kate, Chaturbhuj Rathore, Ashalatha Radhakrishnan

Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India

Correspondence Address: Ashalatha Radhakrishnan, Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India, ashalatharadhakrishnan@gmail.com

Date of Acceptance: 01-Feb-2010

Code Number: ni10086

PMID: 20508366

DOI: 10.4103/0028-3886.63779

Sir,

A 42-year-old lady presented with progressive gait ataxia, intermittent headache, blurring of vision and impaired hearing of one year duration. In addition, relatives noticed brief stereotypic movements of both upper limbs with behavioral arrest and transient unresponsiveness lasting less than a minute occurring at a frequency of one per week since three months prior to admission. She was on phenytoin 300mg/day. No significant past or family history. Neurological examination revealed bilateral papilledema, diminished sensation in the right fifth cranial nerve distribution, bilateral sixth nerve palsy, and sensory-neural hearing loss. Deep tendon reflexes were brisk with extensor plantar response. She had tandem gait ataxia.

MRI showed a heterogeneously enhancing calcified mass lesion (3.5 x 2.7 x 4 cm) in the lateral aspect of fourth ventricle with secondary hydrocephalus [Figure - 1]. A 16-channel-scalp EEG showed bilateral intermittent slowing with persistent photo paroxysmal response (PPR) at 1Hz with intermittent photic stimulation (IPS) with no other epileptiform abnormalities [Figure - 2]. Following external ventricular drainage (EVD), her paroxysmal events subsided. As isolated PPR at 1 Hz is an atypical and uncommon finding, the EEG was repeated after one week, which did not reveal any PPR [Figure - 3]. The patient subsequently underwent resection of the mass lesion (ependymoma) and shunting.

PPR is a highly heritable trait characterized by spikes, spike-waves or intermittent slow waves in response to visual stimulus at various frequencies. ^[1] It is a frequent feature of idiopathic generalized epilepsy (IGE), visual sensitive epilepsy(occipital epilepsy) and in certain childhood epilepsies like severe myoclonic epilepsy in infancy. ^[2] In all these instances, PPR usually becomes evident between 10-20 Hz frequency. ^[3] PPR at 1Hz is a rarity and is usually reported in progressive epileptic encephalopathies like neuronal ceroid-lipofuscinosis (NCL) and mitochondrial cytopathy. Even in normal individuals where PPR occurs as a genetic trait, it occurs at a faster frequency and to the best of our knowledge has not been reported at 1 Hz frequency.

Very few studies have looked into the pathophysiologic basis of PPR. These studies noted " wave-length-dependent PPR′′ and ′′quantity-of-light dependent PPR′′ in IGE, localization-related epilepsy and symptomatic generalized epilepsy. ^[4] It is hypothesized that membrane depolarization of some of the neurons to light stimuli, especially over the posterior head region contribute to photosensitivity, but the exact molecular mechanism underlying PPR remains unknown. Antiepileptic drugs like valproate and levetiracetam are known to suppress PPR, but our patient was not on any of these drugs. We postulate that external ventricular drainage for the hydrocephalus reduced the intracranial pressure and presumably the aberrant neuronal depolarization to photic stimuli related to raised intracranial pressure also subsided.

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