General Hospital Psychiatry 37 (2015) 275.e1–275.e3 Contents lists available at ScienceDirect General Hospital Psychiatry journal homepage: http://www.ghpjournal.com Case Report Psychosis in primary angiitis of the central nervous system involving bilateral thalami: a case report Sangha Kim, M.D., Doh Kwan Kim, M.D., Ph.D. ⁎ Department of Psychiatry, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea a r t i c l e i n f o Article history: Received 14 August 2014 Revised 10 March 2015 Accepted 10 March 2015 Keywords: Primary angiitis Central nervous system Vasculitis Thalamus, Psychosis a b s t r a c t Objective: To report a case of primary angiitis of the central nervous system (PACNS), a rare inflammatory disease restricted to the central nervous system (CNS), with unusual clinical presentation mimicking schizophrenia. Method: Case report. Results: A 45-year-old male presented with alteration of consciousness and confusion. Brain magnetic resonance imaging (MRI) scan showed a mass-like enhancing lesion involving bilateral thalami, and biopsy revealed findings compatible with PACNS. The patient was treated with corticosteroids. Psychotic symptoms crystallized over the initial 2 months after the diagnosis and persisted for over a year. Severity of his symptoms improved with gradual normalization of the radiologic findings and antipsychotic medication. Conclusion: Our case highlights the importance of considering PACNS as a differential diagnosis of a tumor-like mass lesion in the CNS and the significance of thalamic involvement in the pathogenesis of psychotic symptoms including delusions and hallucinations. © 2015 Elsevier Inc. All rights reserved. 1. Introduction Primary angiitis of the central nervous system (PACNS) is a rare inflammatory disease restricted to the central nervous system with variable clinical presentations and radiologic findings [1]. Thalamic involvement has been implicated in the neural basis of schizophrenia [2,3]. Neuropsychological, behavioral, and mood disruptions have also been reported in isolated cases with thalamic infarcts [4–8]. Here, we report a case of mass-like PACNS involving bilateral thalami presenting as schizophrenia-like psychosis in previously healthy male. 2. Case Presentation A 45-year-old hypertensive man presented with alteration of consciousness and confusion following 2 weeks of flu-like symptoms. Neuropsychological evaluation revealed profound deficits in attention, memory, visuospatial, and frontal executive functions. He scored 9/30 on Korean mini mental state examination (K-MMSE). No focal neurological deficits were noted except for mild dysarthria. Psychotic or affective symptoms were not evident. Blood tests were nonspecific except for a slight increase in the erythrocyte sedimentation rate (ESR=25 mm/hr). Cerebrospinal fluid (CSF) findings were absent for malignant cells or elevated white blood cell count. CSF culture was sterile. Brain MRI was notable for hyperintense T2 fluid-attenuated inversion recovery ⁎ Corresponding author. Tel.: +82-2-3410-3582; fax: +82-2-3410-0941. E-mail address: paulkim@skku.edu (D.K. Kim). http://dx.doi.org/10.1016/j.genhosppsych.2015.03.006 0163-8343/© 2015 Elsevier Inc. All rights reserved. (FLAIR) and T1-enhancing mass-like lesion involving bilateral thalami (Fig. 1A, B, D). Navigation-guided biopsy in the right thalamus revealed no evidence of malignancy but multifocal infarction and hemorrhage, perivascular lymphocytic infiltration and intraluminal obliteration of a vessel, suggestive of PACNS. Immunohistochemical studies revealed the infiltrates to be composed of lymphocytes positive for leukocyte common antigen, CD3 and CD20. Fludeoxyglucose positron emission tomography (FDG PET) scanned after the biopsy revealed slight decrease in FDG uptake of bilateral thalami and a focal decrease of FDG uptake in the right frontal lobe and anterior portion of the right thalamus (Fig. 1C). Given the diagnosis of PACNS, the patient was injected with dexamethasone 5 mg two to four times a day for 8 days and was subsequently put on oral prednisolone 30 mg a day for 12 days. He was tapered off oral prednisolone over the following 36 days. The patient was referred to the psychiatric clinic for delusions and irritability 4 months after discontinuation of corticosteroids. He had been paranoid, irritable and violent. His psychiatric symptoms had gradually crystallized over 2 months after the first diagnosis. He believed that the doctors have revived his mother and left razors and scissors in his head. He complained that the furniture was infested with insects. He had delusions of theft and infidelity. He was hostile to his spouse and overtly hypersexual. He was amnestic for memories of recent years and had difficulty acquiring new information. But he watched baseball games on TV and had recently begun to drive his car with frequent mistakes in directions. He was no longer dysarthric. Brain MRI scanned 50 days prior to the visit to our clinic showed decrease in overall extent of the enhancement in bilateral thalami with remaining lesion prominent in the anterior and medial portions of the thalamus (Fig. 1E). He was put on 5 mg of olanzapine a day. 275.e2 S. Kim, D.K. Kim / General Hospital Psychiatry 37 (2015) 275.e1–275.e3 Fig. 1. (A, B, C, & D) January 2013: (A) Axial T2-weighted FLAIR image; (B) axial and (D) coronal enhanced T1-weighted images showed enhancing mass-like lesion involving bilateral thalami; (C) FDG PET image showed slight decrease in FDG uptake of bilateral thalami and focal decrease in FDG uptake in the right frontal lobe and anterior portion of the right thalamus; (E) May 2013: Axial enhanced T1-weighted image showed decrease in overall extent of the enhancement in bilateral thalami; (F) January 2014: Axial enhanced T1-weighted image showed resolution of vasculitis involving both thalami. For the following 1 year, he was titrated up to 12.5 mg and down to 2.5 mg of olanzapine a day due to somnolence. He gradually became less paranoid, less irritable and rarely violent, although he had intermittent bouts of misconception or paranoid ideation. His total positive and negative syndrome scale score decreased from 78 to 58 in a year largely attributable to reduction in positive scales scores. His global cognitive function improved with K-MMSE scores of 20/30 to 29/30. However, he seemed to have lost interest and lacked spontaneity. He was no longer hypersexual and slept for more than 10 hours a day. He had difficulty recognizing nuances in conversations and seemed to have lost empathic abilities. He was put on methylphenidate up to 10 mg/day with improvement in alertness but no significant change in volition. Brain MRI scanned 1 year after the diagnosis showed resolution of vasculitis involving both thalami (Fig. 1F). He was no longer psychotic but grossly abulic and amnestic. 3. Discussion PACNS is a rare disease with limited knowledge about its etiology. PACNS presenting as a mass lesion comprises only about 5% of this rare disease entity [9]. Because there is no specific radiologic finding that could distinguish PACNS from other mass lesions, biopsy remains the gold standard for the diagnosis [9,10]. Once the diagnosis is confirmed, current treatment of choice is either corticosteroids or cyclophosphamide or both. The clinical features of PACNS are variable with headache, altered cognition and neurologic deficits being the most frequent features [1]. Extensive bilateral thalamic lesion in our case has caused a wide range of cognitive, neuropsychiatric and behavioral symptoms. Thalamic dysfunction has been implicated in the pathogenesis of schizophrenia [3]. Structural abnormalities especially in the anterior, dorsomedian nuclei and pulvinar and metabolic abnormalities both in the thalamus and prefrontal regions are observed in schizophrenia [2,3]. Review of isolated cases of thalamic infarctions has also revealed distinct behavioral patterns according to territories [5,6,8]. Lesions in the anterior and paramedian territories mimic psychiatric illnesses with wide range of symptoms including delirium, personality change, frontal syndrome and psychosis presumably due to their rich connections to frontal and limbic structures [2,8]. It is of note that our case presented with no particular motor or sensory disturbances except for mild dysarthria and visual hallucinations early in the course suggesting that the primary sensory and motor relay centers in the thalamus were largely undisturbed. Frontal and anterior thalamic hypometabolism in the initial FDG PET and the resolving patterns of the lesion in T1weighted images as depicted in Fig. 1 point towards the significance of anterior and paramedian regions of the thalamus and their connections to frontal and limbic cortices in the pathogenesis of psychotic symptoms as well as amnesia and abulia in this patient. The patient also presented with an increased level of sexual activities early in the course. Studies have reported hypersexuality in patients with isolated cases of thalamic infarct or with thalamic dysfunction in the context of Kleine–Levin syndrome which is a cyclic hypersomnia associated with symptoms of hyperphagia, hypersexuality and cognitive impairment [11]. To our knowledge, there has been no report of mass-like PACNS involving bilateral thalami presenting as schizophrenia-like psychosis. This case is important in two aspects. First, PACNS, a rare disease entity that still needs clarification regarding etiology, diagnosis and treatment, may present with psychotic symptoms and mood disturbances. Second, it adds to the significance of thalamic involvement in the pathogenesis of psychotic symptoms. Acknowledgement The authors thank Professor Jung Il Lee (Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine) for helpful comments. S. Kim, D.K. 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