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Year : 2019  |  Volume : 41  |  Issue : 2  |  Page : 184-186  

Creutzfeldt–Jacob disease with psychiatric presentation: Hen's teeth in Indian subcontinent: A case report

Department of Psychiatry, Government Medical College and Hospital, Chandigarh, India

Date of Web Publication4-Mar-2019

Correspondence Address:
Dr. Shivangi Mehta
Department of Psychiatry, Government Medical College and Hospital, Sector 32, Chandigarh - 160 047
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPSYM.IJPSYM_102_18

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How to cite this article:
Singh G, Mehta S, Kumar M, Salhotra A. Creutzfeldt–Jacob disease with psychiatric presentation: Hen's teeth in Indian subcontinent: A case report. Indian J Psychol Med 2019;41:184-6

How to cite this URL:
Singh G, Mehta S, Kumar M, Salhotra A. Creutzfeldt–Jacob disease with psychiatric presentation: Hen's teeth in Indian subcontinent: A case report. Indian J Psychol Med [serial online] 2019 [cited 2020 Feb 19];41:184-6. Available from:


Creutzfeldt–Jacob disease (CJD) is a rare, neurodegenerative, prion disease with an incidence of one per million individuals per year and mortality of 100%.[1] It occurs mostly in sporadic form (90%) followed by familial (15%) and acquired forms (5%).[2] Progressive dementia, visual disturbances, myoclonus, extrapyramidal and pyramidal signs, and behavioral disturbances are among the most common clinical findings. Diagnostic criteria of probable sporadic CJD (University of California San Francisco UCSF, 2007)[3] include (1) rapid cognitive decline; (2) at least two of the following six specific neurological manifestations: myoclonus, pyramidal/extrapyramidal, visual, cerebellar, akinetic mutism, other higher cortical signs (e.g., neglect, aphasia, apraxia, acalculia); (3) positive electroencephalography (EEG) (periodic epileptiform discharges) or positive magnetic resonance imaging (MRI) (either subcortical hyperintensity or cortical gyral hyperintensity [cortical ribboning] on DWI (diffusion-weighted imaging) and preferably restricted diffusion on ADC (apparent diffusion coefficient) map); (4) routine investigations do not suggest an alternative diagnosis. Clinical onset is characterized in most cases by neurological symptoms, whereas in a much smaller percentage by signs of mental deterioration and psychiatric symptoms. The differential diagnosis of psychosis in elderly patients usually includes delirium, dementia, and primary psychiatric disorders. A diagnosis of CJD should not be neglected in elderly patients presenting with recent onset and rapid progression of behavioral changes, anxiety, irritability, mood deflection, and insomnia with no psychopathological history.[4] Mehndiratta et al. reported 10 cases of CJD from North India where abnormal behavior was seen in 70% cases.[5] Satishchandra et al. in their epidemiological study recorded 20 definite and 10 probable cases of CJD from 1971 to 1990. In that paper, the authors found that psychiatric manifestations were present at onset in 105 cases and there was clustering of cases from Mumbai and Bengaluru.[6]

   Case Report Top

A 50-year-old woman was brought to the psychiatric outpatient department with history of psychomotor agitation and behavioural problems for the last one and a half months. The personal and family psychiatric history was unremarkable. Initially, the history was taken from the patient's son, who reported that in the last one and a half months the patient had shown behavioural changes, wandering behaviour, and verbal and physical aggression. There was a recent decline in the personal and social functioning. At first psychiatric evaluation, the patient was conscious but confused, not oriented in time, partly oriented towards the place, and seemed uncooperative. Her speech was spontaneous but irrelevant and nongoal directed. Due to her psychomotor agitation, higher mental functions could not be assessed. The patient was admitted, and a provisional diagnosis of a manic episode was kept based on the clinical findings of over-talkativeness, overfamiliarity, agitation, and aggression. Routine blood tests and ECG showed no pathological alterations. To manage the psychiatric symptoms, the patient was started on risperidone (titrated up to 3 mg/day) and diazepam (titrated up to 10 mg/day). Detailed evaluation in the ward revealed memory impairment for the last 3 months. During hospitalization, the patient's condition deteriorated. Perseveration and hallucinatory behavior became evident. A detailed neuropsychological assessment could not be done as the patient was uncooperative. According to the clinical picture, negative psychopathologic history, the rapid progression of memory impairment, and worsening of psychiatric symptoms, an organic substrate for the psychiatric symptoms was hypothesized. Because there was a rapid evolution of symptoms, it was fairly possible to exclude common forms of dementia, such as Alzheimer's disease and frontotemporal dementia. Prion disease was kept as one of the differential diagnoses because of the rapid evolution of symptoms.[7] Neurological symptoms eventually became prominent, with the evolution of apraxia and focal neurological deficits (pyramidal/extrapyramidal signs and akinetic mutism). There was rigidity in all the extremities and hyper-reflexic deep tendon reflexes. MRI brain showed bilaterally symmetrical T2-weighted and fluid attenuated inversion recovery (FLAIR) hyperintensity along the cortex, bilateral globus pallidi, and posteromedial thalami, as well as diffusion restriction and hockey stick appearance of thalami [Figure 1] and [Figure 2].
Figure 1: MRI Brain showing cortical ribboning on DWI

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Figure 2: MRI brain showing diffusion restriction in bilateral posteromedial thalami giving the hockey-stick appearance

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EEG revealed bilateral periodic sharp and short interval diffuse discharges with generalized delta slowing. Cerebrospinal fluid (CSF) analysis was not done as the diagnosis was achieved with the help of neuroimaging and EEG findings. There was worsening of psychiatric symptoms despite treatment and the patient was lost to follow-up.

   Discussion Top

CJD is a rare, fatal neurodegenerative disease with distinctive clinical and pathological features. Sporadic CJD (sCJD) is the most common form of spongiform encephalopathy and usually appears at 50-70 years of age, consisting 90% of all cases with CJD.

Wall et al. retrospectively reviewed 126 patients of sporadic CJD in Mayo Clinic from 1976 to 2001, and found that 26% of the patients had psychiatric symptoms at presentation while 80% demonstrated some psychiatric dysfunction within 100 days of onset of illness. Prominent psychiatric symptoms were sleep disturbances, psychosis, and depression.[4]

The diagnosis of sCJD is based on clinical symptoms along with laboratory tests including EEG, imaging, and CSF analysis for 14-3-3 protein.[1] On T2-weighted MRI, hyperintense areas could be found in the caudate nucleus, putamen, globus pallidus, and thalamus. Diffusion-weighted MRI may indicate bilateral symmetrical hyperintense signals in the basal ganglia and/or cortex cerebri. Shiga et al. reported sensitivity and specificity of diffusion-weighted MRI in diagnosing CJD as 92.3% and 93%, respectively.[8] Cerebral atrophy can be another MRI finding. 14-3-3 protein is a protein released from damaged neurons into CSF, and is a good marker for CJD, with the sensitivity and specificity reported near 94% and 84%, respectively.[9] EEG is a diagnostic modality with sensitivity near 64% and specificity between 74 and 91%.[8]

Our patient presented with a mania-like episode and not discrete episodes of psychosis, which makes it an unusual presentation of CJD to be kept in mind.

   Conclusion Top

In clinical practice, CJD should not be neglected as a differential diagnosis in adult and elderly patients with negative psychiatric history referred to psychiatrists for recent onset and rapidly progressing symptoms such as behavior changes, anxiety, irritability, mood deflection, insomnia, and poor response to treatment. In this case, the symptoms and signs necessary to diagnose a possible sporadic form of CJD emerged only belatedly, at a final stage, while symptoms at onset appeared to be more behavioral in nature.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Weihl CC, Roos RP. Creutzfeldt-Jacob disease, new variant Creutzfeldt-Jacob disease and Bovine spongiform encephalopathy. Neurol Clin North Am 1999;17:835-59.  Back to cited text no. 1
Masters CL, Harris JO, Gajdusek DC, Gibbs CJ Jr, Bernoulli C, Asher DM. Creutzfeldt- Jacob disease: Patterns of worldwide occurrence and significance of familial and sporadic clustering. Ann Neurol 1979;5:177-88.  Back to cited text no. 2
Available from: [Last accessed on 2018 Jan 01].  Back to cited text no. 3
Wall CA, Rummans TA, Aksamit AJ, Krahn LE, Pankratz VS. Psychiatric manifestations of Creutzfeldt-Jakob disease: A 25-year analysis. J Neuropsychiatry Clin Neurosci 2005;17:489-95.  Back to cited text no. 4
Mehndiratta MM, Bajaj BK, Gupta M, Anand R, Tatke M, Seryam S, et al. Creutzfeldt- Jakob disease: Report of 10 cases from North India. Neurol India 2001;49:338-41.  Back to cited text no. 5
[PUBMED]  [Full text]  
Satishchandra P, Shankar SK. Creutzfeldt-Jakob disease in India (1971-1990). Neuroepidemiology 1991;10:27-32.  Back to cited text no. 6
Gozke E, Erdal N, Unal M. Creutzfeldt-Jacob disease: A case report. Cases J 2008;1:146.  Back to cited text no. 7
Zerr I, Pocchiari M, Collins S, Brandel JP, de Pedro Cuesta J, Knight RS. Analysis of EEG and CSF 14-3-3 proteins as aids to the diagnosis of Creutzfeldt-Jakob disease. Neurology 2000;55:811-5.  Back to cited text no. 8
Shiga Y, Miyazawa K, Sato S, Fukushima R, Shibuya S, Sato Y, et al. Diffusion weighted MRI abnormalities as an early diagnostic marker for Creutzfeldt-Jacob disease. Neurology 2004;63:443-9.  Back to cited text no. 9


  [Figure 1], [Figure 2]


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