J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.47.9.1050 on 1 September 1984. Downloaded from

1050 Letters embedding it remains feasible that the two of three objects after five minutes. or the major sign of an exacerbation.36 virus that caused encephalitis lethargica (if There were no clear-cut cognitive deficits Gilmore et a13 reported a patient with virus it was) may yet be identified in mater- noted. known sarcoidosis thought to be in remis- ial that has been stored since the time of Outpatient treatment with imipramine sion who had a depressive episode without the epidemic. was begun for major depression. This other evidence of active sarcoidosis. The therapy was discontinued because of into- cerebrospinal fluid was suggestive of active MM ESIRI lerable lethargy. The patient was then disease. In Hook's4 report of nine cases of The Radcliffe Infirmary, admitted to the inpatient psychiatric unit neurosarcoidosis, case number 2 was diag- Oxford OX26 6HE for electroconvulsive therapy. At that time nosed as depression. Zerman6 described a her mental status examination was mixed psychiatric-neurologic syndrome M SWASH unchanged and the remainder of her with features of Wernicke-Korsakoff London Hospital, neurological exam was reported as normal. psychosis as the initial manifestation of sar- Whitechapel, After eight treatments (one bilateral and coidosis. Suchenwirth and Dold5 described London UK seven unilateral) she became severely con- a man with a paranoid psychosis who was fused with urinary incontinence and found to have sarcoidosis. His psychiatric difficulty in walking. Neurological consul- symptoms responded to steroid medica- tation was requested. On examination, she tion. References was disoriented to time, place, and could Our patient initially had no clear evi- not identify her psychiatrist. There were no dence of active sarcoidosis. The clinical pic- 'Flexner S. Epidemic (lethargic) encephalitis motor or sensory abnormalities other than ture of and allied conditions. JAMA 1923; tearfulness, constricted affect, 81:1688-93; 1785-9. a slightly wide-based, unsteady gait. An anhedonia, and disturbance of sleep and 2 Levaditi C, Harvier P, Nicolam P. Etude erythematous malar rash was noted. Inves- appetite supported a diagnosis of a major exp6rimentale de l'enc6phalitie dite 'ldthar- tigation of the cause of her encephalopathy depressive episode. Following ECT, the gique'. Ann Inst Past 1922;36:63-148. showed cerebrospinal fluid (CSF) to con- profound organic mental syndrome, gait 3Esiri MM. Herpes simplex encephalitis: an tain protein 160 mg/dl, glucose 40 mg/dl, disorder, and urinary incontinence indi- immunohistological study of the distribution 22 WBC/mm3 with 83% lymphocytes, cated the organic basis of the disease and of viral antigen within the brain. J Neurol 15% monocytes, 2% polymorphonuclear prompted the neurologic consultation. TheProtected by copyright. Sci 1982;54:209-26. cells, negative VDRL, negative bacterial diagnosis of was on 4 Kennard C, Swash M. Acute viral encephalitis: neurosarcoidosis based its diagnosis and outcome. Brain and fungal cultures, and no malignant cells. the positive skin biopsy, positive Kveim 1981; 104:129-48. An electroencephalogram showed test and cerebrospinal fluid formula of ele- increased diffuse activity in the theta and vated protein, low glucose, and a mild, delta frequencies. The CT scan showed predominantly lymphocytic pleocytosis. As Neurosarcoidosis presenting as major decreased attenuation in the white matter with Suchenwirth and Dold's5 case and depression adjacent to the lateral ventricles most other patients with dementia due to sar- prominently in the frontal region, and a coid,2 our patient had a favorable clinical Sir: Neurological complications of sar- cerebral angiogram was normal with no response to steroid medication with coidosis occur in approximately 5% of all evidence of vasculopathy. Blood chemistry significant improvement of the CSF cases.' Rarely, dementia mimicking CBC, ANA, ESR, and thyroid function abnormalities. Alzheimer's disease may be the presenting studies were all normal. The CSF findings The CT scan showed decreased attenua- or prominent manifestation of the illness.2 from a second lumbar puncture were: pro- tion of the periventricular white matter, Less commonly, prominent psychiatric tein 139 mg/dl, glucose 46 mg/dl, 21 WBC/ primarily in the frontal region. The rele- manifestations in relative isolation can mm3 with 83% lymphocytes, 14% mono- vance of this is not clear. CT findings in occur at the onset or during an exacerba- cytes, 3% polymorphonuclear cells, and no intracranial neurosarcoidosis are non- tion of the disease.23 We describe a patient malignant cells. A skin biopsy taken in the specific. They include normal scans, hyd- who ultimately proved to have neurosar- area of the malar rash showed non- rocephalus, abnormal enhancement of the coidosis and whose clinical presentation caseating granulomata. Kveim-Siltzbach meninges (especially in the basal area), dif- was indistinguishable from a major depres- antigen was injected and the biopsy several fuse enhancement throughout the entirehttp://jnnp.bmj.com/ sive episode. weeks later was positive for sarcoidosis. cerebrum and intraparenchymal mass A 57-year-old chronically dysphoric There was no definitive evidence of sarco- lesions which often have a somewhat woman complained to her psychiatrist of idosis on several chest radiographs. increased density compared to brain tissue deteriorating memory and concentration The patient was treated with corticos- and show enhancement on contrast which forced her to give up her job as an teroids (prednisone 60 mg/day). Within studies.''3 The association of sarcoidosis office manager. Initial examination by her three weeks of starting treatment her think- and progressive multifocal leukoence- psychiatrist showed her to be oriented and ing was more lucid and her mood brighter. phalopathy is known.' In our patient this coherent but tearful, with depressed mood CSF findings at that time were: protein 68 possibility cannot be entirely ruled out. and constricted affect. Her thought content mg/dl, glucose 70 mg/dl, 9 WBC/mm3 with However, it is unlikely because of the on October 1, 2021 by guest. included feelings of helplessness, hopeless- 81% lymphocytes, 9% monocytes, 10% improvement in clinical status and CSF ness, anhedonia, and complaints of insom- polymorphonuclear cells. A repeat CT scan abnormalities with steroids. In addition, nia and anorexia causing a 10 pound (4.5 was unchanged. the usual CT findings of low density lesions kg) weight loss. She named the past four There are four reported cases in the lit- of the central and convolutional white mat- presidents of the USA, spelled a four letter erature of sarcoidosis in which a psychiatric ter with scalloped lateral borders were not word forwards and backwards, and recalled syndrome was the presenting manifestation present.'4 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.47.9.1050 on 1 September 1984. Downloaded from

Letters 1051 These cases illustrate that the principal inflammation and focal hydrocephalus clinical manifestations of sarcoidosis may shown by sequential CT. Neurol (Minneap) 1979;29: 1161-65. on rare occasions be psychiatric and 12 Kendall BE, Tatler GLV. Radiological findings emphasise the importance of the possibility in neurosarcoidosis. Br J Radiology of effective treatment with steroids. 1978;51:81-92. 3 Brooke SB, El Hammal T, Hungerford GD, We are grateful to Dr Stefan Shanzer for Acker J, Trevor RP, Russel W. Radiologic review of this manuscript. evaluation of neurosarcoidosis: role of computed tomography. Am J Neuroradiol- JOHN STILLER* ogy 1982;3:513-21. Caroll BA, Barton L, Norman D, Engmann D. ANDREW GOODMAN* Diagnosis of progressive multifocal LAWRENCE M KAMHIt leukoencephalopathy by computed tomog- MICHAEL SACHER* raphy. Radiology 1977; 122:137-41. MORRIS B BENDERO Accepted 17 March 1984 The Departments ofNeurology, * Psychiatry, t and Radiology, t The Mount Sinai Medical Center, New York, and The Mount Sinai School ofMedicine ofthe City University ofNew York. Address for reprint requests: Dr John Stil- ler, Department of Neurology, 2150 Penn- sylvania Avenue, NW Washington, DC 20037, USA §deceased Protected by copyright.

References Delaney P. Neurologic manifestations in sar- coidosis: review of the literature with a report of 23 cases. Ann Intern Med 1977;87:336-45. 2 Cordingly G, Navano C, Brust JCM, Healton ET. Sarcoidosis presenting as dementia. Neurol (NY) 198 1; 31: 1148-51. 3Gilmore K, Rudden M, Kalman TP. Psychiatric manifestations of sarcoidosis. Can J Psychiatry 1980; 25:329-31. Hook 0. Sarcoidosis with involvement of the nervous system. Report of nine cases. Arch Neurol Psychiatry 1954;71:554-75. 5Suchenwirth R, Dold V. Functional psychoses in sarcoidosis. Verh Dtsch ges Inn Med 1969;75:757-59. 6 Zerman W. Die meningoencephalitis Besnier-Boeck-Schuumann. Nervenartz http://jnnp.bmj.com/ 1952;23:43-52. 7Morehouse H, Danziger A. CT findings in intracranial neurosarcoid. Comput Tomogr 1980;4:267-70. 8Kumpe DA, Rao-Krishna CVG, Garcia JH, Heck AF. Intracranial neurosarcoidosis. J Comput Assist Tomogr 1979;3: 324-30. Babu VS, Eisen H, Pataki K. Sarcoidosis of the central nervous system. J Comput Assist Tomogr 1979;3: 396-7. on October 1, 2021 by guest. 0 Brooks J, Strickland MC, Williams JP, Vulpe M, Fowler FL. Computed tomography changes in neurosarcoidosis clearing with steroid treatment. J Comput Assist Tomogr 1979;3:398-9. Ho SU, Berenberg RA, Kwang KS, DalCanto MC. Sarcoid encephalopathy with diffuse