Psychosomatics 40:524-525, December 1999
© 1999 The Academy of Psychosomatic Medine
Creutzfeldt-Jakob Disease Presenting as Secondary Mania
Ivan Lendvai , M.D.,
Stephen M. Saravay , M.D., and
Maurice D. Steinberg , M.D.
Received October 15, 1998; revised May 3, 1999; accepted May 20, 1999. From the Long Island Jewish Medical Center, Consultation-Liaison Psychiatry, New Hyde Park, New York. Address correspondence and reprint requests to Dr. Lendvai, Staten Island University Hospital, Department of Psychiatry, 375 Seguine Avenue, Staten Island, NY 10309.
Key Words: Creutzfeldt-Jakob Disease • Mania
Ours is a report of a patient with Creutzfeldt-Jakob disease who presented with mania and was initiallly diagnosed and treated for Bipolar I Disorder, manic type. Psychiatric disturbances constitute the prodromal manifestations in 18%–39% of those with Creutzfeldt-Jakob disease.1 Dementia occurs in all patients and progresses rapidly. Patients may complain of fatigue and appear apathetic; personal hygiene suffers early; in some cases irritability may be prominent.2,3 Depression has been found in more than 30% of patients with Creutzfeldt-Jakob disease, and 10% of patients with Creutzfeldt-Jakob disease need psychiatric hospitalization for depression.1,2 We were unable to find any report of mania as a prominent presenting symptom.
Case Report
The patient, a 45-year-old, married mother of two, was in her usual state of health, working as a secretary until about 8 weeks before admission to a short-term psychiatric inpatient facility. At that time, the patient began to have pressured, incoherent speech, with thoughtracing, and abrupt shifts of thoughts. She went on spending sprees and built up considerable credit card debt, buying unnecessary things. She had severe insomnia, sleeping only a few hours each night. She also complained of blurred vision and gait difficulty, the latter also noted by her family. After evaluation of these complaints and a normal magnetic resonance imaging (MRI) of the brain, she was given a diagnosis of Bipolar I Disorder, manic type. After 2 weeks, she was discharged on Haldol (haloperidol: 15 mg/day), Cogentin (benztropine: 0.5 mg bid), and Depakote (divalproex sodium: 750 mg bid). During the first week at home, she became less spontaneous, increasingly lethargic, and less interpersonally responsive, and her gait problems worsened. She spent much of her time staring into space, not speaking. During the second week at home, the patient became increasingly agitated. Her medications were stopped; Klonopin (clonazepam) was started without improvement, and the patient was hospitalized at another acute psychiatric hospital, again diagnosed as Bipolar I Disorder, manic type.
Within 72 hours of admission, the patient became completely nonverbal, and she was transferred to the Long Island Jewish Medical Center and admitted to the neurology service. A psychiatric consultation was called to evaluate whether the mental status change might have been caused primarily by a psychiatric disorder.
Prior medical history was insignificant, except for high blood pressure and a hysterectomy secondary to a fibroid tumor. Medications included estrogen patches and Vasotec (enalapril maleate). There was no history of mental illness, smoking, or alcohol or drug abuse. The family history was significant in that the patient's father committed suicide, her mother had syphilis, and a brother had a history of heroin overdose. On mental status examination, the patient was found lying in her bed, with her eyes open, staring into space. She was unresponsive to questions. No further examination was possible.
Test results: lactate dehydrogenase 826 U/L. Other laboratory tests were normal (complete blood cell count, serum Na [sodium], serum K [potassium], glucose, creatinine, total bilirubin, albumin, alkaline phosphatase, cholesterol, aspartate aminotransferase, alanine aminotransferase, urine analysis). Test for syphilis was nonreactive, and serum ammonia was within normal limits. Thyroid functions were normal. The neurology examination: central nerves II–XII intact. There was no motor or sensory deficit. Gait could not be tested becase the patient was bedridden. The cerebrospinal fluid was normal. Toxicology screen: negative. A computed tomography scan of the head without contrast was unremarkable.
An electroencephalogram (EEG) taken on the day of admission was later reported as showing evidence of a severe disturbance of cortical activity. Triphasic sharp wave complexes were present, consistent with metabolic and toxic encephalopathy as well as certain degenerative conditions (Creutzfeldt-Jakob). After admission, acyclovir (700 mg q 8 hr IV) was started.
The patient was continuously monitored with videocamera and electroencephalography during Days 4–9. Because there appeared to be a quantitative improvement over the 4 days (the periodic phenomena remained, but occupied a smaller percentage of the total), a potentially reversible encephalopathy seemed more likely than a progressive degenerative disorder such as Creutzfeldt-Jakob disease.
A diagnosis of encephalopathy of unknown origin was made, and the psychiatric consultation-liaison service was called again to transfer the patient back to psychiatry, because of persisting lethargy. On examination the patient was found to be unresponsive to verbal stimuli, with eyes open, staring into space. She was noted to have jerky, myoclonic limb movements. Further diagnostic workup to determine etiology was suggested in view of her worsening clinical condition and the newly noted myoclonic movements.
Neurology reevaluation confirmed the presence of myoclonic movements induced by startle reactions; she also had a rigid neck and increased reflexes; and clonic, spastic muscle tone and jerky movements in all extremities. A clinical diagnosis of spongo-encephalopathy, Creutzfeldt-Jakob disease was made. Contact isolation was ordered. Brain MRI was unsuccessful because of the patient's agitation. An EEG on Day 20 was qualitatively similar to the earlier recordings, with some decrease in the triphasic sharp wave complexes.
Because of the discrepancy between the worsening clinical picture and the stable or improving EEG, a sample of cerebrospinal fluid was sent to the National Institutes of Health for prion protein study. A modified SDS-PAGE (sodium dodecylsulfate-polyacryamide gel electrophoresis) with immunoblotting of the sample was done: protein 130/131 was present. This assay among demented patients has a sensitivity of 96% and specificity of 99%.4 A diagnosis of Creutzfeldt-Jakob disease was made. The patient's condition gradually deteriorated, and she died 2 months after admission.
Discussion
Creutzfeldt-Jakob disease is a type of subacute spongiform encephalopathy caused by transmissible agent termed a prion. In the case of Creutzfeldt-Jakob disease we have described, prominent symptoms of mania, including pressured speech, thoughtracing, abrupt shift of thought, insomnia, and spending sprees building up considerable credit card debt, were the presenting symptoms. .A psychiatric diagnosis Bipolar I Disorder, manic type was made. In our case, the suicide of the patient's father and the unusual appearance of manic symptoms in Creutzfeldt-Jakob disease raise the question of whether a familial predisposition to bipolar disorder may have been present that became manifest in our patient with the onset of her Creutzfeldt-Jakob disease. However, we were unable to obtain any history of prior affective illness in the patient, or in other family members, or manic episodes in her father.
Secondary mania consists of manic symptoms associated with antecedent physical illness or drug use. Secondary mania can occur with physical illness;5 medications (e.g., bronchodilators, corticosteroids); metabolic disturbances (e.g., vitamin B12 deficiency, hemodyalisis thyrotoxicosis); neoplasm central nervous system diseases (e.g., Parkinson disease, cerebritis due to lupus, multiple sclerosis, epilepsy, cerebrovascular accident); and infections (e.g., neurosyphilis, HIV [human immunodeficiency virus infection], cryptococcosis, influenza), all which can cause secondary mania.6–8 Lyketsos et al. in a chart review found that 8% of the patients who had AIDS (acquired immunodeficiency syndrome) were affected with manic symptomes.9 Our case demonstrates that mania may also be a presenting symptom of Creutzfeldt-Jakob disease and that the disorder should be considered in the differential diagnosis of organic brain disorders that may present with manic symptoms.
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