Neurosarcoidosis occurs in 3 to 10% of patients with sarcoidosis. The granulomatous infiltration of the central nervous system (CNS) may produce a wide variety of mental symptoms. Psychiatric manifestations occur in 20% of patients with neurosarcoidosis.
A 21-year-old female patient presented to the psychiatric outpatient department with a history of hyperactivity in the form of over-talkativeness, over-familiarity, aggression, hypersexuality, fearfulness, and persecutory delusions for 1 month. Cognitive symptoms in the form of loss of attention and concentration were also reported. She sometimes complained of having a unilateral left-sided headache. Her physical examination was unremarkable. On neurological examination, the reflexes were 2+ (brisk) and symmetric at the biceps, triceps, knees, and ankles. There was no dysmetria on the finger-to-nose and heel-to-shin tests. Also, new skin lesions, lymphadenopathy, or asterixis were absent on physical examination. A provisional diagnosis of mania with psychotic features was made on the history and physical examination. The young mania rating scale was administered, and the score came up to 39. The patient was diagnosed with mania with psychotic symptoms as per ICD classification and started on multiple antipsychotics and mood stabilizers with no significant improvement.
The patient did not respond to the treatment, and to rule out any organic causes, neuroimaging of the brain was performed. The magnetic resonance imaging (MRI) of the brain showed hyperintensities on the dorsal medulla, suggesting neurosarcoidosis, which led to further testing [Figure 1]. Baseline investigation was typical with mild eosinophilia (8%). Her thyroid profile was also within the normal range. The serum angiotensin-converting enzyme (ACE) levels were mildly elevated, and cerebrospinal fluid (CSF) analysis was carried out (mentioned in Table 1. The antinuclear antibodies were positive, which led to further testing. Consultation with a neurologist was advised with pulse steroid therapy trial of 3 months with monitoring of side effects. Flunarizine was used for headache, tablet albendazole, and injection of methylprednisolone 1 g for 3 days every month for the next 3 months as a treatment option.
Sarcoidosis of the CNS has been variously reported in 5–15% of all sarcoid patients. The patient in our care was unique because her diagnosis was made in the psychiatry department.
We describe the case of a 21-year-old female patient with reported symptoms (mentioned above) because of which she was hospitalized in the psychiatric department. Her brain MRI indicated hyperintensities in the dorsal medulla, suggesting neurosarcoidosis. This is a rare case of neurosarcoidosis, replete with mania and cognitive impairments.
A diagnosis of mania with psychotic characteristics was made based on the psychological evaluation imagery. Neurosarcoidosis has been linked to a variety of cognitive and behavioral symptoms such as refractory psychosis, hallucinations, paranoid psychosis, and delirium. Cognitive impairments such as nominal aphasia, amnestic syndrome, and dementia have been reported. Schizophreniform condition and other mental disorders, including fast-cycling disease, major depressive disorder, and bipolar disorder, have to be observed. Patients with sarcoidosis with psychiatric symptoms should be evaluated for neurosarcoidosis as corticosteroid treatment can only improve symptoms. There has been many reports on atypical presentation of neurosarcoidosis with some common neuropsychiatric manifestations listed in Table 2. The caveat is that in a patient with multisystem sarcoidosis, an unexplained mental deterioration should be an indication for aggressive evaluation of the CNS.
Neurosarcoidosis may be misdiagnosed if the psychological and cognitive behavioral changes appear as the first manifestations of the disease. Physicians should evaluate the likelihood of active CNS involvement in individuals with psychotic symptoms and cognitive deficits.
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1. Ungprasert P, Matteson EL. Neurosarcoidosis. Rheum Dis Clin North Am 2017;43:593–606.
2. Om P, Sharma AMS. Sarcoidosis of the nervous system. Arch Intern Med 1991;151:1317–21.
3. Spiegel DR, Thomas CS, Shah P, Kent KD. A possible case of mixed mania due to neurosarcoidosis treated successfully with methylprednisolone and ziprasidone:Another example of frontal- subcortical disinhibition?. Gen Hosp Psychiatry 2010;32:342 e1–3 doi:10.1016/j.genhosppsych.200.907.010.
4. Vannemreddy PS, Nanda A, Reddy PK, Gonzalez E. Primary cerebral sarcoid granuloma:The importance of definitive diagnosis in the high-risk patient population. Clin Neurol Neurosurg 2002;104:289–92.
5. Bona JR, Fackler SM, Fendley MJ, Nemeroff CB. Neurosarcoidosis as a cause of refractory psychosis:A complicated case report. Am J Psychiatry 1998;155:1106–8.
6. Hayashi T, Onodera J, Nagata T, Mochizuki H, Itoyama Y. [A case of biopsy-proven sarcoid meningoencephalitis presented with hallucination, nominal aphasia and dementia. Rinsho Shinkeigaku 1995;35:1008–11.
7. O'Brien GM, Baughman RP, Broderick JP, Arnold L, Lower EE. Paranoid psychosis due to neurosarcoidosis. Sarcoidosis 1994;11:34–6.
8. Bourgeois JA, Maddock RJ, Rogers L, Greco CM, Mangrulkar RS, Saint S. Neurosarcoidosis and delirium. Psychosomatics 2005;46:148–50.
9. Willigers H, Koehler PJ. Amnesic syndrome caused by neurosarcoidosis. Clin Neurol Neurosurg 1993;95:131–5.
10. Friedman SH, Gould DJ. Neurosarcoidosis presenting as psychosis and dementia:A case report. Int J Psychiatry Med 2002;32:401–3.
11. Sabaawi M, Gutierrez-Nunez J, Fragala MR. Neurosarcoidosis presenting as schizophreniform disorder. Int J Psychiatry Med 1992;22:269–74.
12. Walbridge DG. Rapid-cycling disorder in association with cerebral sarcoidosis. Br J Psychiatry 1990;157:611–3.
13. Stiller J, Goodman A, Komhi LM, Sacher M, Bender MB. Neurosarcoidosis presenting as major depression. J Neurol Neurosurg Psychiatry 1984;47:1050–1.
14. McLoughlin D, McKeon P. Bipolar disorder and cerebral sarcoidosis. Br J Psychiatry 1991;158:410–3.
15. Hoitsma E, Faber CG, Drent M, Sharma OP. Neurosarcoidosis:A clinical dilemma. Lancet Neurol 2004;3:397–407.