Late-onset sporadic ataxia, pontine lesion, and retroperitoneal fibrosis: a case of Erdheim-Chester disease.
Neurol Sci
; 29(4): 263-7, 2008 Sep.
Article
in En
| MEDLINE
| ID: mdl-18810602
ABSTRACT
A 60-year-old man with progressive gait ataxia and mild pyramidal signs showed at MRI a pontine lesion with post-contrast enhancement in the left middle cerebellar peduncle. Diagnosis of Erdheim-Chester disease (ECD), a rare non-Langerhans cell histiocytosis, was suggested, further supported by a previously diagnosed retroperitoneal fibrosis. X-ray films demonstrated characteristic bilateral and symmetric osteosclerosis of the long bones of the lower limbs, which at radionuclide studies exhibited a marked increase in technetium-99 uptake. A cerebral 18FDG-PET showed a relevant pontine uptake of the tracer. Re-evaluation of a past retroperitoneal biopsy showed an intense CD68+, CD1a-, and S100- infiltrate of histiocytes with foamy cytoplasm, thus confirming the diagnosis. ECD should be regarded as a rare cause of adult-onset sporadic ataxia, especially when pontine lesions and extraneurological manifestations are present.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Retroperitoneal Fibrosis
/
Pons
/
Cerebellar Ataxia
/
Erdheim-Chester Disease
Type of study:
Diagnostic_studies
/
Etiology_studies
Language:
En
Journal:
Neurol Sci
Journal subject:
NEUROLOGIA
Year:
2008
Document type:
Article
Affiliation country:
Italy
Publication country:
IT
/
ITALIA
/
ITALY
/
ITÁLIA