Dural Sarcoidosis Presenting With Transient Neurologic Symptoms Kapil D. Sethi, MD, MRCP; Taher El Gammal, MD; Bipin R. Patel, MD; Thomas R. Swift, MD \s=b\ Despite the propensity of sarcoid granulomas to be clustered around blood vessels, transient ischemic attacks and strokes are rare. A 43-year-old man had recurrent transient dysarthria and right hemiparesis; a biopsy specimen showed neurosarcoidosis. There was complete resolution of both symptomatology and the subdural sarcoid mass lesion with the administration of corticosteroid therapy. (Arch Neurol 1986;43:595-597) involvement occurs in "neurologie of sarcoid¬ 2% to 16%l of the cases osis, either from space-occupying or infiltrating granulomas or from gran¬ ulomas infiltrating the blood ves¬ sels.2'5 Pathologically, infarctions oc¬ cur in the territory of involved ves¬ sels, but the clinical occurrence of transient ischemie attacks (TIAs) and strokes is rare.6 We describe a patient who had recurrent focal neurologic deficits resembling classic TIAs due to neurosarcoidosis. REPORT OF A CASE A 43-year-old man had been examined elsewhere three years earlier for urinary incontinence and weakness and stiffness of the legs. A total axis myelogram was nor¬ mal then. Cerebrospinal fluid contained oligoclonal bands and an elevated protein Accepted for publication Nov 6, 1985. From the Departments of Neurology (Drs Sethi, Patel, and Swift) and Radiology (Dr El Gammal), Medical College of Georgia, Augusta. Reprint requests to the Department of Neurology, Medical College of Georgia, Augusta, GA 30912 (Dr Sethi). level. He was believed to have a demyeiin¬ ating disease and was treated with a brief course of corticosteroids (dosage and dura¬ tion unknown) without any response. Two years later, he began having recurrent episodes of weakness and numbness of the right side of the face and body usually lasting 30 to 60 minutes. The maximum duration recorded was 12 hours. These stereotyped attacks consisted of numbness of the arm or leg spreading within seconds to involve the entire right side of the body along with muscle weakness and slurred speech. This was often followed by throb¬ bing bifrontal headache without nausea or vomiting. There were no visual distur¬ bances preceding or accompanying these episodes. Examination during one of the transient episodes revealed the patient to be alert. He had fluent paraphasic speech and diffi¬ culty with naming, repetition, and compre¬ hension. There was weakness of the lower portion of the right side of the face and right arm with brisk reflexes. Legs were spastic with bilateral Babinski's signs. Findings from a sensory examination were normal. A general physical examination showed no abnormalities. At the time of his initial admission to our hospital, a computed tomographic (CT) head scan (with and without contrast enhancement), cerebral angiogram, electroencephalogram (EEG), echocardiogram, and Holter electrocardio¬ graphic monitoring were normal. Formal ophthalmologic examination revealed no abnormality. Aspirin therapy produced no improvement in the patient's condition. He was readmitted to the hospital briefly eight months later and a CT scan and echocardiogram were again normal. A CT head scan one year after onset of symptoms showed an enhancing lesion in the extracerebral space over the left pari¬ etal region (Fig 1). Retrospective analysis Fig 1.—Computed tomographic scan with contrast enhancement showing enhancing lesion (arrow) over left parietal region. of the scan done four months earlier revealed that the same lesion was present, though much less distinctly. Cerebral angi¬ ography showed displacement of the distal branches of the left middle cerebral artery away from the inner table of the skull and an elliptic avascular space consistent with an extracerebral collection (Fig 2). Selec¬ tive external carotid showed no abnormali¬ ty. An EEG showed mild slowing in the left anterior and midtemporal region that was made worse by drowsiness. The cerebrospi¬ nal fluid protein level was elevated to 72 mg/dL, glucose level was 52 mg/dL, and there were four lymphocytes per cubic millimeter in the cerebrospinal fluid. Electrophoresis revealed a polyclonal in¬ crease in the globulins without a discrete oligoclonal band. The VDRL test was nega- Downloaded From: http://archneur.jamanetwork.com/ by a Michigan State University User on 06/17/2015 Fig 2.—Left carotid angiogram shows avascular extracerebral lesion (double arrow) with displacement of branches of middle cerebral artery Fig 3.—Histologie specimen shows multiple granulomas with epithelioid cells and giant cells. branches away from inner table of skull. tive. Cultures for bacteria, acid-fast bacilli, and fungi were negative. A repeated myelogram showed only mild spondylosis at C5-6 bilaterally. A chest roentgenogram was normal. Angiotensin-converting en¬ zyme was 133 units (normal, 40 to 125 units). A left parietal burr hole biopsy was performed; the specimen revealed noncaseating granulomas with an abundance of histiocytes and Langhans' and foreignbody giant cells (Fig 3). Special stains for bacteria, acid-fast bacilli, and fungi were negative. With prednisone treatment, 60 mg/day, the episodes stopped. A follow-up CT scan done five months later showed complete resolution of the enhancing mass seen on earlier scans. Prednisone therapy was gradually tapered to 10 mg every other day. One year later he had two additional TIAs. A CT scan showed recurrence of the left extracerebral mass. Prednisone dosage was increased to 60 mg/day, again result¬ ing in complete cessation of the episodes. A follow-up CT scan showed diminution in the size of the extracerebral mass. There has been no change in the spasticity of the legs and the patient is still bound to a wheelchair. COMMENT Sarcoid involvement of the nervous system usually manifests itself as cra¬ nial (primarily facial) and peripheral neuropathies, granulomatous menin¬ gitis, and hypothalamic-posterior pi¬ tuitary axis involvement.6 Rarer man¬ ifestations include myelopathy,' en¬ cephalopathy, and intracranial mass lesions.89 When the neurologic mani¬ festations appear in the setting of established systemic disease, diagno¬ sis usually is easy. Sarcoidosis may be limited to the cranial nervous sys¬ tem,51011 however, and when nervous system involvement is the initial and/ or only manifestation, misdiagnoses are common. Subdural location of sarcoid tissues is rare; to our knowledge, there are only two previous reports of subdural Downloaded From: http://archneur.jamanetwork.com/ by a Michigan State University User on 06/17/2015 sarcoid granulomas.1213 How the sub¬ dural collection of sarcoid granu¬ lomas was related to transient focal deficit in our patient is unclear. Com¬ plicated migraine cannot be ruled out, but the typical march of paresthesia14 or weakness was absent, and the patient had no visual disturbances. A seizure is unlikely because there was no involuntary jerking of the extremi¬ ties preceding the paresis and the EEG during one episode showed no seizure discharge. It is unlikely to be due to the mass effect because the episodes had started long before the subdural mass was large enough to be seen on CT scan. These attacks resem¬ bled TIAs clinically, though the angio¬ gram failed to reveal involvement of larger vessels. Transient focal neurologic deficits and strokes have only rarely been attributed to sarcoidosis.15·16 Transient ischemie attacks have been reported to be the sole manifestation of neuro¬ sarcoidosis, but no convincing sam¬ ples are given.17 Sarcoid cardiomyopathy may cause cerebral embolism.18 Histopathologically, however, involvement of blood vessels and perivascular tissues occurs in brain and meninges and may produce subclinical infarctions.24 The process mainly affects small vessels, but no vessel is exempt.5 One of us (T.E.G.) previously reported angiographically evident arteritis in a patient with neurosarcoidosis, who, however, did not have TIAs or stroke.19 Corticosteroids are the treatment of choice for intracranial sarcoid mass lesions. Surgery should be reserved for biopsy or for relief of rapidly increasing intracranial pressure. The excellent outcome in this patient treated with corticosteroids contrasts sharply with the other published case in which the subdural collection was removed surgically.12 It is likely that the myelopathy in this patient also was due to neurosar¬ coidosis. Myelography may be normal in sarcoid myelopathy.20 In neurosar¬ coidosis, increased cerebrospinal fluid 7-globulin levels occur frequently20·21 and oligoclonal bands have been described.22 We propose that neuro¬ sarcoidosis should be considered in the differential diagnosis of TIAs, particularly in young patients, as this is a potentially treatable disorder. References 1. Silverstein A, Feuer MM, Siltzback LE: Neurologic sarcoid: Study of 18 cases. Arch Neu- rol 1965;12:1-11. 2. Urich H: Neurological manifestations of sarcoidosis. Practitioner 1969;202:632-636. 3. Aszkanazy CL: Sarcoidosis of the central nervous system. J Nuropathol Exp Neurol 1952;11:392-400. 4. Meyer J, Foley J, Campagna Pinto-D: Granulomatous angiitis of the meninges in sarcoidosis. Arch Neurol 1953;69:587-600. 5. Reske E, Nielsen AA, Harmsen GE: Periangiitis and panangiitis as a manifestation of sar- coidosis of the brain: Report of a case. J Nerv Ment Dis 1962;135:399-412. 6. Delaney P: Neurologic manifestations of sarcoidosis. Ann Intern Med 1977;87:336-345. 7. Day AL, Sypert GW: Spinal cord sarcoidosis. Ann Neurol 1977;1:79-85. 8. Powers WJ, Miller EM: Sarcoid mimicking glioma: Case report and review of intracranial sarcoid mass lesions. Neurology 1981;31:907-910. 9. Brooks BS, Gammal TE, Hungerford GD, et al: Radiologic evaluation of neurosarcoidosis: Role of computed tomography. AJNR 1982;3:513\x=req-\ 521. 10. Zeman W: Morbus Besnier-Boeck Schaumann, in Handbuch der Speziellen. Pathologischen Anatomie and Histologie, 1958;XIII/2: 1100-1112. 11. Douglas AC, Maloney FJ: Sarcoidosis of the central nervous system. J Neurol Neurosurg Psychiatry 1973;36:1024-1033. 12. 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Caroscio JT, Yahr MD: Progressive myelopathy due to sarcoid. Clin Neurol Neuro- 1975;293:1138\x=req-\ surg 1980;82:217-222. 21. Colover S: Sarcoidosis with involvement of nervous system. Brain 1948;71:451-475. 22. Kinnman T, Link H: Intrathecal production of oligoclonal IGM and IGG in CNS sarcoidosis. Acta Neurol Scand 1984;69:97-106. Downloaded From: http://archneur.jamanetwork.com/ by a Michigan State University User on 06/17/2015