Neurosarcoid

Case contributed by Dr Dalia Ibrahim

Presentation

Headache, dizziness, bilateral upper and lower limb weakness.

Patient Data

Age: 35 years
Gender: Female

MRI and MR spectroscopy

MRI

Multiple leptomeningeal and parenchymal enhancing nodules are seen at the suprasellar/hypothalamic region, right temporal lobe, and left frontal regions. They elicit isointense signal at T1 WI, a low signal at T2 WI with homogenous post-contrast enhancement. No diffusion restriction. They are surrounded by vasogenic edema and exert a positive mass effect. Thickening and enhancement of the pituitary infundibulum. Left frontal subcortical white matter focus of abnormal signal with no contrast enhancement.

MRS criteria were non-specific, with no significant elevation of the Choline peak. No significant reduction of the NAA peak.

MRI of the cervical spine

MRI

The cervical cord opposite C2 shows a small anterior peripherally located intramedullary enhancing nodule similar to the previously noted cerebral lesions.

Both MRI and MRS of the brain as well as MRI of the cervical cord were highly suggestive of neurosarcoid. Other possibilities included CNS lymphoma and granulomatous infection (TB).

The patient denied sarcoidosis or any systemic disease, she underwent CT scan of the chest.

CT scan of the chest

CT

Multiple pathologically enlarged bilateral hilar, right paratracheal, and subcarinal lymph nodes, Characterstic of pathological nodal enlargment in Sarcoidosis.

Lung window revealed left upper lobar and to less extent right middle and lower lobar small nodules surrounded by ground-glass attenuation, giving the "halo sign". Bilateral lower lobar subpleural patches of ground-glass attenuation and interstitial thickening.

Case Discussion

In this case, neurological symptoms were the first manifestation and the patient was imaged without the diagnosis of systemic sarcoidosis.

MRI & MRS criteria of the cerebral parenchymal, leptomeningeal, and cervical cord lesions were highly suggestive of neurosarcoid.

Subsequent CT scan of the chest proved typical features of pulmonary sarcoidosis with pathologically enlarged bilateral hilar, paratracheal and subcarinal lymph nodes, as well as multiple patches of ground-glass attenuation.

The patient later had positive laboratory data for sarcoidosis with increased serum and CSF ACE levels. Increased serum amyloid A protein (SAA). Normal Parathyroid hormone and calcium levels.

Neurosarcoid could involve the following:

  • skull vault involvement, in the form of lytic lesions, mimicking metastasis.
  • pachymeningeal thickening of low signal on T2 WI with homogenous contrast enhancement.
  • leptomeningeal involvement, which is either focal or generalized, nodular or smooth, and has a predilection to basal aspects and around circle of Willis. Leptomeningeal nodules may extend to the brain via perforating vessels and perivascular spaces, like in this case.
  • pituitary and hypothalamic involvement
  • cranial nerve involvement
  • parenchymal involvement (most common), likely via extension from leptomeningeal disease via perivascular space, likely in the form of nodules or masses.

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