[Atypical presentation of intramedullary sarcoidosis: report of two cases].
BACKGROUND
Neurological involvement in sarcoidosis occurs in 5 to 15% of cases, but medullary involvement is very uncommon.


METHODS
We report two cases of sarcoidosis with medullary involvement as an initial presentation. Case 1: a 41 year-old man presented with a four-month history of lumbar pain, gait and sphincter disturbances; examination revealed a spastic paraparesis, hyperreflexia and a sensory level by D4-D6. Serum angiotensin converting enzyme (SACE) was normal. Magnetic resonance image (MRI) showed diffuse spinal cord enlargement with hyperintensity signal between D4-D5 and D10-11, with contrast enhancement. Spinal biopsy was consistent with sarcoidosis. Case 2: a 36 year-old man presented with a two-months history of gait difficulties and two episodes of blurred vision on his left eye that improved with topical therapy. Examination revealed lupus pernio in the ears and a spastic hyperreflexic paraparesis. Laboratory detected increased SACE. MRI showed focal spinal cord areas of hyperintensity at cervico-dorsal levels with contrast enhancement. Ear biopsy was consistent with sarcoidosis. Clinical courses under corticosteroids were favourable in both cases.


CONCLUSIONS
Intramedullary lesions are infrequently the first manifestation of sarcoidosis especially when there is no systemic involvement (case 1). It is also rare in association with lupus pernio and ocular involvement (case 2). In the later (with systemic manifestations) SACE was raised. Biopsy is very useful for early diagnosis.
Perdigão
S
S
Unidad de Neurología, Hospital Pedro Hispano, Senhora da Hora, Matosinhos, Portugal. sandraperdigao@mail.pt
Arantes
M
M
Pinheiro
L
L
Costa
M
M
D000328:Adult; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008526:Medulla Oblongata / Q000473:pathology*; D009422:Nervous System Diseases / Q000175:diagnosis / Q000473:pathology; D012507:Sarcoidosis / Q000175:diagnosis / Q000473:pathology