Research Day
Is It Conversion disorder or Creutzfeldt jakob disease, Psychiatric presentation
Document Type
Abstract
Date
2017
Abstract
Introduction: Cruestzfeldt Jakob disease is a form of nervous and mental illness associated with progressive central nervous system degeneration, first described by Creutzfeldt and Jakob in the early 1920s. CJD principally affects the grey matter of cerebral cortex, brainstem and molecular layer of cerebellum. It is believed to be caused by histologically unconventional infectious agents called PRIONS. It is uniformly fatal and the annual incidence rate is 1-2 per million worldwide. Most cases are sporadic, but 5-15% of cases have a genetic basis and have been clustered within families, inherited as an autosomal dominant trait with variable penetrance. Despite isolated case reports of accidental human to human transmission of CJD (following human growth hormone therapy, corneal transplantation, brain transplantation and contaminated EEG electrode implantation. Researchers believe that direct inoculation of the agent remains the only proven mechanism of transmission. Forty-one percent of the cases begin as gradually progressive mental deterioration evidenced by psychiatric disturbances; 36% initially present with nonspecific neurological symptoms only; and the remaining 23% of cases present with both neurological and psychiatric prodromal features. With the exception of brain biopsy, there are no specific tests for CJD. Here we report a case of CJD with a complicated initial presentation which led to an initial possible diagnosis of Conversion disorder and psychiatric admission. Patient presented with rapid decline in vision which was presumed to be conversion disorder, as patient had a significant history of neurotic disorders. Case description: A 73 year old married female who was referred by her primary care physician to the Emergency department for further evaluation of poor vision and personality changes. Patient’s initial symptoms began over a course of 4-5 months, starting in July 2014, where she was noted to be forgetful and “clumsy”, bumping into furniture while walking. Her family noticed significant changes from being a happy and outgoing person to recently describe as withdrawn and irritable. She was an exceptional “knitter” but noted be undexterous and buttered finger. She had a stressful relationship with her daughter, whom she hasn’t seen in years. Pt had a history of depression which was diagnosed in June 2006 close to her retirement. She was being treated with SSRI, Sertraline and treated intermittently with Alprazolam for anxiety. Pt had developed few panic attacks in the context of significant stresses with her step children and therefore treated with alprazolam. She had no history of panic attacks or conversion in the past. By October 2014 patient’s symptoms had progress and now she was experiencing staring blankly into space and occasional flinging movements of her arms and legs. These symptoms were ignored by the family as they believed “her throwing a tantrum”. During this time patients daily activities became increasingly chaotic. She was seen by her primary care where she was prescribed an increase dose of antidepressant along with scheduled dose of anxiolytic. Her husband noted that patient complained of vision disturbances and was referred to an ophthalmologist by her primary care. She was seen by an ophthalmologist twice who failed to find any ocular pathology but documented visual losses by perimetry testing. On 13th November patient was referred to Emergency room by her ophthalmologist as she had left hemianopia and complete loss in her right eye. Psychiatry was consulted from the ER where patient had an initial CT scan and basic labs which were non revelatory. Finally patient was admitted under psychiatry service with a differential diagnosis of 1) Conversion disorder; 2) Adjustment disorder or atypical depression. On mental status exam patient was cognitively compromised and appeared to be significantly depressed. Once again, complete laboratory workup was unremarkable. Physical exam showed poor visual acuity and posturing in upper extremity. Pt was given 2mg Ativan IM for possible conversion disorder but showed no effect. Patient was deteriorating, incoherent, agitated and depressed. She repeatedly said” there is no point”. EEG was grossly abnormal and revealed periodic lateralizing epileptiform discharges (PLEDS). Pt was transferred to ICU for non-convulsive status epilepticus. CSF studies were unremarkable. Tentative diagnosis of encephalopathy was made, CSF was sent for 14-3-3 protein and NMDA receptor antibody. Her hospital course was rapidly downhill until her death in December 2014. Discussion: This case illustrates patient with CJD, whose complexity of prodromal presentation obscured the early diagnosis. The major factors that led the physicians to consider alternative psychiatric diagnosis during early stages of illness were: 1) history of depression; 2) stressors and variability of neurological symptoms, reinforcing the possibility of primary psychiatric illness. We conclude that psychiatric symptoms are common in CJD and many times precede the onset of neurological deficits and raise the suspicion for a neurologic rather than a psychiatric etiology.