Letters to the Editor

Pankaj Gandhi Department of Neurology, Sawai ManSingh Medical College Hospital, Jaipur, Rajasthan, India For correspondence:

Dr. Rajendra Singh Jain, 126‑Janakpuri‑II, Imli Phatak, Jaipur,

Rajasthan, India. E‑mail: [email protected]

References 1. Miller DH, Robb SA, Ormerod IE, Pohl KR, MacManus DG, Kendall BE, et al. Magnetic resonance imaging of inflammatory and demyelinating white‑matter diseases of childhood. Dev Med Child Neurol Suppl 1990;32:97‑107. 2. Hergüner MÖ, Altunbaşak Ş, Baytok  V. Patients with acute, fulminant form of SSPE. Turk J Pediatr 2007;49:422‑5. 3. Dyken PR. Subacute sclerosing panencephalitis. Current status. Neurol Clin 1985;3:179‑96. 4. Mahadevan A, Vaidya SR, Wairagkar NS, Khedekar D, Kovoor JM, Santosh V, et al. Case of fulminant‑SSPE associated with measles


genotype D7 from India: An autopsy study. Neuropathology 2008;28:621‑6. 5. PeBenito R, Naqvi SH, Arca MM, Schubert R. Fulminant subacute sclerosing panencephalitis: Case report and literature review. Clin Pediatr (Phila) 1997;36:149‑54. 6. Garg RK. Subacute sclerosing panencephalitis. Postgrad Med J 2002;78:63‑70. 7. Gökçil Z, Odabaşi Z, Aksu A, Vural O, Yardim M. Acute fulminant SSPE: Clinical and EEG features. Clin Electroencephalogr 1998;29:43‑8. 8. Alexander M, Singh S, Gnanamuthu C, Chandi S, Korah IP. Subacute sclerosing panencephalitis: CT and MRI imaging in a rapidly progressive case. Neurol India 1999;47:304‑7. Access this article online Quick Response Code:

Website: www.annalsofian.org

DOI: 10.4103/0972-2327.120437

Moyamoya disease with exaggerated startle response: A rare co‑occurrence Dear Sir, Moyamoya disease  (MMD) presenting with movement disorders in the form of chorea, dystonia, and diskinesias has been reported before; however, MMD with exaggerated startle response has not been reported previously.[1,2] Recently, we came across a case of MMD featuring exaggerated startle response. A 7‑years‑old boy, presented with the sudden jerky movements of body in the form of blink, flexion of neck and trunk, and abduction of arm especially in response to loud noise since the age of 6 years [Video 1]. It often resulted in fall and injuries. There was no history of loss of consciousness with these jerks. He had past history of recurrent strokes at the age of 3 years. No other family members or siblings were affected with similar illness. At the time of presentation he was conscious, alert, and was able to understand commands. His speech was limited to speaking bi‑syllables only. Residual right hemiparesis with spasticity was present. Involuntary movement in the form of exaggerated startle response to sudden, loud noise was present. No spontaneous involuntary movements were observed. Computed tomography  (CT) scan of head showed bilateral cerebral hemisphere infarcts in middle cerebral artery territory. He was diagnosed to have MMD on the basis of angiographic findings which showed occlusion of bilateral supraclinoid internal carotid arteries and proximal middle cerebral arteries with bilateral basal collaterals as well as right posterior cerebral artery  (P1) stenosis [Figure 1].

Figure 1: Computed tomography angiography brain showing occlusion of bilateral supraclinoid internal carotid arteries and proximal middle cerebral arteries with collaterals at the base of the brain suggestive of moyamoya disease along with stenosis of P1 segment of right posterior cerebral artery

Exaggerated startle syndromes (hyperekplexia) consist of an excessive motor response or jump, to unexpected auditory, somesthetic, and visual stimuli. An exaggerated startle syndrome may be due to local brainstem pathology (anoxia, inflammatory lesions, and hemorrhage) and also occurs as an inherited condition transmitted as an autosomal‑dominant trait.[3] Our patient presented with recurrent ischemic strokes at the age of 3 years and exaggerated startle response since the age 6 years [Video 1]. Development of exaggerated startle response in this patient can be explained by the ischemia of brainstem with the progression of moyamoya

Annals of Indian Academy of Neurology, October-December 2013, Vol 16, Issue 4


Letters to the Editor

vasculopathy involving posterior circulation. Jayakumar, et al.,[4] in their series reported that posterior circulation is frequently involved in MMD as evident by angiography; ischemic events of the posterior circulation are not frequent, as the posterior circulation acts as collateral pathway for the diseased anterior circulation till the later stage. Mugikura, et al., [5] reported high prevalence of clinical strokes and infarctions in patients diagnosed before 4 years of age, associated with advanced steno‑occlusive lesions of the posterior cerebral artery (PCA). To conclude, this is the first report of sporadic exaggerated startle syndrome  (sporadic hyperekplexia) in association with MMD. This case suggests that moyamoya syndrome should be considered in differential diagnosis of exaggerated startle response especially on the background of strokes in childhood.

Rajendra Singh Jain, Rahul Jain, Tarun Mathur, B.S Raghavendra

Rajasthan, India. E‑mail: [email protected]

References 1. Gonzalez‑Alegre P, Ammache Z, Davis PH, Rodnitzky RL. Moyamoya induced paroxysmal dyskinesia. Mov Disord 2003;18:1051‑6. 2. Baik JS, Lee MS. Movement disorders associated with moyamoya disease: A report of 4 new cases and review of literatures. Mov Disord 2010;25:1482‑6. 3. Fahn S, Jankovik J, editors. Principles and Practice of Movement Disorders. Philadelphia: Churchill Livingstone; 2007. p. 527‑8. 4. Jayakumar PN, Vasudev MK, Srikanth SG. Posterior circulation abnormalities in moyamoya disease: A radiological study. Neurol India 1999;47:112‑7. 5. Mugikura S, Higano S, Shirane R, Fujimura M, Shimanuki Y, Takahashi S. Posterior circulation and high prevalence of ischemic stroke among young pediatric patients with moyamoya disease: Evidence of angiography‑based differences by age at diagnosis. AJNR Am J Neuroradiol 2011;32:192‑8. Access this article online Quick Response Code:

Department of Neurology, Sawai ManSingh Medical College, Jaipur, Rajasthan, India

Website: www.annalsofian.org

DOI: 10.4103/0972-2327.120439

For correspondence:

Dr. Rajendra Singh Jain, Department of Neurology, Sawai Man Singh Medical College, Jaipur ‑ 302 004,

A rare presentation of methanol toxicity Dear Sir, We read with great interest the article entitled “A rare presentation of methanol toxicity” by Gupta et al.,[1] Indeed the presentation of methanol toxicity can be variable, with high anion gap metabolic acidosis, visual abnormalities, obtundation, and neurological deficit often associated with death. We also came across a case recently, which was associated with acute onset blindness with encephalopathy after consumption of methanol wherein MRI findings showed bilateral putaminal hemorrhagic necrosis. Patient, however, showed remarkable recovery on follow‑up visits. The reported patient here had sudden onset vision loss after consumption of country liquor. Curiously, fundus findings suggestive of presence of any optic atrophy have not been mentioned. Also the possible explanation of sluggishly reacting pupils in the above patient is not clear. It would have been extremely helpful if the value of P100 latencies on visual evoked response had been mentioned. Also the prolonged latencies were due to demyelination or falsely positive due to cortical blindness the patient was having, has not been clearly ascertained. The patient had infarcts in areas supplied by various vessels and the mechanism given by

authors is cerebral vasospasm but specifically which vessel is involved has not been mentioned, also the lacunae was that, it had not been confirmed by either CT angiography/MR angiography or Digital subtraction angiography. The reported patient is of 51 years of age, so possibility of other vascular risk factors cannot be denied but the authors did not consider either echocardiography or Carotid Doppler in their patient; therefore, looking into the possibility of infarcts involving multiple territory a cardio‑embolic source rather than concomitant country liquor consumption is highly likely. Hence, whether the infarcts were due to methanol intoxication or just an incidental finding is still not clear. The characteristic neuroimaging findings of bilateral hemorrhagic necrosis, cerebral and intra‑ventricular hemorrhage, cerebellar necrosis, and diffuse cerebral edema have been described as sequelae to severe methanol intoxication. Putaminal hemorrhagic necrosis results from direct toxic effect of methanol metabolites and metabolic acidosis. It often has poor prognosis, i.e.,  either death or vegetative state.[2‑4] In a country like ours where easy availability of spurious liquor has been a concern, this article highlighted about the variable presentation of methanol poisoning and that timely institution of treatment can be indeed life‑saving.

Annals of Indian Academy of Neurology, October-December 2013, Vol 16, Issue 4

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Moyamoya disease with exaggerated startle response: A rare co-occurrence.

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