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LETTER TO EDITOR
Year : 2005  |  Volume : 53  |  Issue : 1  |  Page : 120-121

A case of sporadic Creutzfeldt Jakob disease with anterior visual pathway involvement


1 Departments of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum - 695 011, India
2 Departments of Radiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum - 695 011, India

Date of Acceptance08-Nov-2004

Correspondence Address:
P S Mathuranath
Departments of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum - 695 011
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.15080

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How to cite this article:
Mooney T, Sreekumar J, Hemanth S, Mathuranath P S, Sarada C. A case of sporadic Creutzfeldt Jakob disease with anterior visual pathway involvement. Neurol India 2005;53:120-1

How to cite this URL:
Mooney T, Sreekumar J, Hemanth S, Mathuranath P S, Sarada C. A case of sporadic Creutzfeldt Jakob disease with anterior visual pathway involvement. Neurol India [serial online] 2005 [cited 2019 Oct 21];53:120-1. Available from: http://www.neurologyindia.com/text.asp?2005/53/1/120/15080


Sir,

 Creutzfeldt-Jakob disease More Details (CJD) typically presents with rapidly progressing dementia, myoclonus and rigidity,[1] although early cortical blindness is known in the Heidenhain variant.[2] Early and predominant anterior visual pathway involvement is very rare in sporadic CJD.

A 61-year-old previously asymptomatic lady presented with a 3 months history of progressive, painless bilateral visual loss culminating in complete blindness. Behavioral alterations manifested two months later as apathy, decreased word output, and a fluctuating sensorium. Over the next couple of months she developed gait and limb ataxia, and limb dystonia. She had no history of blood transfusion or surgery and was a vegetarian who occasionally consumed fish but never meat or beef. We first saw her in the ninth month of her illness. She was doubly incontinent, had no visual regard, withdrew limbs to painful stimuli, and vocalized spontaneously and to painful stimuli with no meaningful verbalization. Menace and pupillary light reflex (direct and consensual) were absent, optic fundii showed bilateral primary optic atrophy, deep tendon reflexes were normal and plantar response flexor. Over the next four weeks of hospital stay she developed spontaneous as well as action-induced myoclonus with axial and appendicular rigidity.

Serum biochemical tests, thyroid functions, anti-thyroid antibodies, fine needle aspiration cytology and ultrasonography of the thyroid, and serum B12 levels were all within normal limits. HIV and ANA serology were negative. Cerebrospinal fluid (CSF) showed 2 lymphocytes/cumm., sugar of 97 mg/dl and protein of 21 mg/dl. CSF Grams and AFB stains and cultures were negative. Flash VEP recorded prolonged P 100 latencies bilaterally (154 msec). The initial three weekly-EEGs showed diffuse slowing with triphasic waves [Figure - 1] while the last EEG showed generalized short interval sharp and slow wave complexes at 1 to 1.5 second [Figure - 2]. Non-contrast (1.5 Tesla) MRI brain showed age-related atrophy and bilateral posterior thalamic hyperintense signal changes on T2 and PD images [Figure - 3].

Our patient started with progressive blindness followed over next ten months by dementia and myoclonus. Other causes were ruled out by appropriate investigations. CJD was considered initially though the optic neuropathy and the absence of the characteristic EEG findings by ten months into the illness raised questions. The subsequent evolution of dystonia, rigidity, myoclonus, and the characteristic periodic complexes on EEG and posterior thalamic hyperintensities on MRI, however, favor the diagnosis of sporadic CJD.[3]

Anterior visual pathway involvement has so far been reported in only two case reports in the literature. Kitagawa et al from Japan reported a patient who had extensive and early brain atrophy on CT scan and whose post mortem showed degeneration of the optic nerve as well as the cerebral and cerebellar white matter.[4] Lesser et al reported a case of bilateral optic atrophy confirmed at autopsy in whom features of optic atrophy were not documented ante mortem.[5] Our case highlights the rare occurrence of the anterior visual pathway involvement in sporadic CJD and demonstrates that it can be the presenting feature, preceding the typical clinical or EEG features of this disease by months.

 
  References Top

1.Wehl CC, Roos RP. Creutzfeldt Jakob disease, new variant Creutzfeldt Jakob disease and Bovine spongiform encephalopathy. Neurol Clin North Am 1999;17:835-59.  Back to cited text no. 1    
2.Kropp S, Schulz-Schaeffer WJ, Finkenstaedt M, Riedemann C, Windl O, Steinhoff BJ, et al. The Heidenhaim variant of Creutzfeldt Jakob disease. Arch Neurol 1999;56:55-61.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.World Health Organisation's Clinical Criteria for the Diagnosis of Sporadic Creutzfeldt Jakob Disease. Wkly Epidemiol Rec 1998;3:361-5.  Back to cited text no. 3    
4.Kitagaway Y, Gotch F, Koto A, Ebihara S, Okayasu H, Ishii T, et al. Creutzfeldt Jakob Disease: A case with extensive white matter degeneration and optic atrophy. J Neurol 1983;229:97-101.  Back to cited text no. 4    
5.Lesser RL, Albert DM, Bobowick AR, O'Brien FH. Creutzfeldt Jakob Disease and optic atrophy. Am J Ophthalmol 1979;87:317-21.  Back to cited text no. 5    


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[Figure - 1], [Figure - 2], [Figure - 3]



 

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