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Meningeal Sarcoidosis, Pseudo-Meningioma, and J Neurol Neurosurg Psychiatry: First Published As 10.1136/Jnnp.55.4.300 on 1 April 1992

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Meningeal Sarcoidosis, Pseudo-Meningioma, and J Neurol Neurosurg Psychiatry: First Published As 10.1136/Jnnp.55.4.300 on 1 April 1992 30030ournal ofNeurology, Neurosurgery, and Psychiatry 1992;55:300-303 Meningeal sarcoidosis, pseudo-meningioma, and J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.55.4.300 on 1 April 1992. Downloaded from pachymeningitis of the convexity D Ranoux, B Devaux, C Lamy, J Y Mear, F X Roux, J L Mas Abstract lb) and was enhanced on TI-weighted gado- Two cases of meningeal sarcoidosis with linium MRI (fig 1c). Carotid angiography unusual and misleading presentations are showed a slight stenosis of the C5 segment of reported. In the first case, CT scan, the left internal carotid artery. Selective left angiographic, and MRI findings were external carotid angiography showed a vas- indistinguishable from those ofmeningio- cular lesion lateral to the carotid siphon, ma. CSF pleiocytosis may help in diag- supplied by the middle meningeal artery (fig nosing sarcoid pseudo-meningioma. The id). Cerebrospinal fluid was normal except for second patient had transient focal deficits elevated proteins (0-85 g/l). A diagnosis of and pachymeningitis ofthe convexity. The meningioma was made, and the patient was transient deficits were probably of epi- treated with carbamazepine. leptic origin based on their response to She was readmitted 16 months later because antiepileptic treatment. The diagnosis of of worsening headaches and diplopia. Exam- neurosarcoidosis was made only after ination showed trigeminal hypesthesia, ptosis meningeal biopsy, despite thorough and paresis ofthe medial rectus on the left. CT investigations. scans and MRI showed an increase in the size of the lesion. A craniotomy revealed a firm, whitish tissue which was adherent to the dura, Neurological symptoms, including cranial extended to the cavernous sinus, and reached neuropathies, aseptic meningitis, hydrocepha- the sphenoid wing. The lesion, grossly resem- lus, intracranial masses, encephalopathies, bling a meningioma, was partially removed. seizures, peripheral neuropathies, and myo- The histological examination showed multiple pathies, develop in 2% to 5% of patients with epithelioid granulomas without necrosis. sarcoidosis.' Symptoms of meningeal involve- Prednisone was increased to 50 mg per day. ment are protean and may take the form of The left ptosis, headaches, and diplopia subacute, chronic, or recurrent meningitis. resolved within a few days. Many patients are asymptomatic.' 2 We report two unusual and misleading CASE 2 presentations of sarcoid meningeal involve- A 48 year old white woman had a four month ment. One mimicked a meningioma, both history of recurrent episodes of left hemipar- http://jnnp.bmj.com/ clinically and radiologically. The other patient esis. These attacks of weakness were of acute had transient focal deficits and pachymeningi- onset, lasted five to 10 minutes, and were tis of the convexity. The diagnosis was made sometimes preceeded by a right temporal only after meningeal biopsy despite thorough headache. They always affected the left arm investigations. and sometimes the left leg. In addition she experienced unrelated attacks of clonic move- ments of her left hand, lasting a few minutes. Case reports Her medical history was unremarkable. On on September 29, 2021 by guest. Protected copyright. CASE 1 admission examination revealed weakness and A 32 year old West Indian woman was admit- astereognosis ofher left hand. An EEG showed ted with a two month history of continuous left paroxymal activity in the right temporal region, sided retro-orbital headache associated with increased by hyperventilation. She received paraesthesia of the left side of her face. phenobarbitone and the attacks stopped. Plain Centre Raymond Pulmonary and ocular sarcoidosis had been CT scan (fig 2a) showed increased density of Garcin, H6pital Sainte diagnosed in 1981, and she was being treated the gyri of the right temporo-parieto-occipital Anne, 1 rue Cabanis, 75674 Paris Cedex 14, with prednisone (100 mg/day) and dapsone and left occipital regions, which enhanced after France (100 mg/day). Neurological examination contrast. There was a large area of hypodensity Service de Neurologie left fifth crani- in the white matter ofthe and D Ranoux revealed hypesthesia around the right hemisphere C Lamy al nerve with decreased corneal reflex and a left left occipital region. TI-weighted MRI showed JY Mear ptosis. The erythrocyte sedimentation rate a slight hypointense signal of the white matter J L Mas (ESR) was 23 mm in the first hour. The with mass effect. On T2-weighted sections the Service de angiotensin-converting enzyme (ACE) activity normal high signal intensity of the sulci was Neurochirurgie B Devaux and chest x ray pictures were normal. A CT replaced by a hypointense signal, which F X Roux scan showed a slightly hyperdense homoge- enhanced after gadolinium injection (fig 2b, c). Correspondence to: neous contrast-enhancing mass lying along CSF examination revealed a white cell count of Professor Mas. side the left cavernous sinus (fig la). This 608 mm3 with 80% lymphocytes, 10% mono- Received 1 May and in lesion was and surrounded by cytes, and 8% a protein content of revised form 4 July 1991. hypointense neutrophils, Accepted 17 July 1991 oedema on axial T2-weighted MR images (fig 1 30 g/l, and a glucose concentration of Meningeal sarcoidosis, pseudo-meningioma, and pachymeningitis of the convexity 301 Figure la CT scan: J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.55.4.300 on 1 April 1992. Downloaded from contrast-enhancing mass (arrow) lying along left cavernous sinus. (b) T2-weighted MRI lesion (arrows) I (2000/90); hypointense compared to nomal brain. (c) Tl-eighted MRI (SE 48115) with injection of gadolinium; enhancement of lesion. (d) Selective external carotid angiography; lesion (arrow) fed by branches of middle meningeal artery. I 1 'I http://jnnp.bmj.com/ on September 29, 2021 by guest. Protected copyright. 1 84 mmol/l. CSF cultures for acid-fast organ- isms and fungi, india ink preparation, and cryptococcal antigen study were negative. The ESR was 23 mm in the first hour. ACE concentration in serum and CSF, tuberculin test (10 units), liver and salivary gland biopsy specimens, thoracic CT scan, fibreoptic bron- Microscopic examination revealed a fibrous choscopy, Gallium scintigraphy, and extensive thickening ofthe meninges and nodular granu- serological studies all yielded normal or neg- lomatous lesions, without central necrosis. ative results. The pulmonary function tests Some follicles had a perivascular distribution. showed a significant decrease in the diffusion The walls of some vessels were invaded by capacity. Despite the negativity of the acid-fast lymphocytes with disruption of the internal organisms cultures, the patient was treated elastic lamina. Cultures for bacteria, acid-fast with antituberculous drugs. organisms, and fungi were negative. The cort- A meningeal and cortical biopsy was per- ical samples showed no abnormality. These formed two months after admission. The dura findings were consistent with the diagnosis of was tense and adherent to the cortex. The sarcoidosis. The patient was treated with pred- brain surface was yellowish and rubbery. nisone (1 mg/kg per day) with continuation of 302 Ranoux, Devaux, Lamy, Mear, Roux, Mas J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.55.4.300 on 1 April 1992. Downloaded from Figure 2a CT scan without contrast; spontaneous increased density and thickening of suki of right temporo-parieto-occipital and left occipital regions. Large hypodensity in white matter. (b) T2-weighted MRI (2200190); large area of hypersignal in white matter with mass effect. Normal signal of sulci replaced by hypointense signal. (c) Tl-weighted images MRI (600115) with injection ofgadolinium; enhancement of suki corresponding to hypointense linear images seen on T2 sequences. addition, selective external carotid angio- first graphy may reveal a blush,8 as seen in our http://jnnp.bmj.com/ case. MRI findings in sarcoid pseudo-menin- gioma have not been reported. In case 1, the lesion was clearly hypointense on T2-weighted sequences and enhanced after admininstration of gadolinium. Meningiomas are usually hyperintense or isointense of T2-weighted sequences but 9% to 18% are hypointense.9 CSF examination may help in diagnosing on September 29, 2021 by guest. Protected copyright. sarcoid pseudo-meningioma when it reveals a pleocytosis,24 which has not been reported in antituberculous therapy. Two months later her meningiomas.10 In case 1, the CSF cell count clinical examination was normal. A CT scan was normal, but the patient was receiving and MRI showed a clear decrease in the size of corticosteroids at the time of the lumbar white matter lesions and the persistance of the puncture. We suggest that patients with a low signal in the affected sulci. known sarcoidosis and evidence of an antracra- nial mass undergo a lumbar puncture (if not contraindicated by the volume of the lesion) Discussion and receive a trial of steroids before surgery. The diagnosis of meningeal sarcoidosis in case Radical removal may be justified only if the 1 was preoperativally mistaken for meningio- lesion is causing important neurological ma. This had also occurred in the few pre- impairment. viously reported cases of sarcoid pseudo- Pachymeningitis of the convexity is a rare meningioma.2-8 Radiological investigations presentation of neurosarcoidosis.1 -16 Head- cannot differentiate between the two diseases. aches are the most common clinical feature In all cases of sarcoid pseudo-meningioma,
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