[A suspicious case of central neurosarcoidosis, manifesting as a progressive gait disturbance with a lesion in the central gray matter of the midbrain, accompanying an elevated level of angiotensin converting enzyme in the cerebrospinal fluid].
We present a suspicious case of central neurosarcoidosis that presented with progressive gait disturbance probably caused by central vestibular dysfunction. And this case showed elevated level of angiotensin converting enzyme (ACE) in the cerebrospinal fluid, compared with the average level of two cases with acute inflammatory demyelinating neuropathy syndrome and four cases of multiple sclerosis. A 33-year-old man was admitted to our hospital with chief complaint of a gait disturbance that had appeared 3 years prior to the admission. And the symptom had exacerbated in these 3 months. Except for the gait disturbance and positive Romberg's sign, no neurological abnormality was detected. The findings of the cerebrospinal fluid test supported the diagnosis of meningitis. An increased level of angiotensine converting enzyme was detected when compared with our previous samplings from two cases of Guillain Barré syndrome and four cases of multiple sclerosis. With T1 weighted imaging of brain MRI, a high intensity lesion with gadolinium enhancement was identified in the central gray matter of the midbrain. Scan of the chest confirmed bilateral hilar lymphadenopathy. Based on these findings and the patient's clinical course, central neurosarcoidosis was suspected. The patient's symptoms improved dramatically after the administration of corticosteroid. The enhancement of the central gray matter ameliorated, and the ACE level of the CSF was decreased to the level of the demyelinating disorders.
Ito
Yasuo
Y
Department of Neurology, School of Medicine, Saitama Medical University.
Nakazato
Yoshihiko
Y
Tomioka
Ryo
R
Tamura
Naotoshi
N
Shimazu
Kunio
K
D000328:Adult; D002493:Central Nervous System Diseases / Q000175:diagnosis* / Q000188:drug therapy; D020234:Gait Ataxia / Q000209:etiology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008636:Mesencephalon / Q000473:pathology*; D007703:Peptidyl-Dipeptidase A / Q000134:cerebrospinal fluid*; D011239:Prednisolone / Q000008:administration & dosage; D012507:Sarcoidosis / Q000175:diagnosis* / Q000188:drug therapy
D011239:Prednisolone; D007703:Peptidyl-Dipeptidase A