Neuro-Behçet disease presenting with acute psychosis in an adolescent.

Behçet disease is a systemic vasculitis of unknown etiology that can affect the neurologic system. Neuro-Behçet disease is not well defined in children and adolescents, and the diagnosis is difficult to make in this population as they often present with insufficient symptoms to meet diagnostic criteria. Psychiatric symptoms as the initial manifestation of neuro-Behçet disease has rarely been reported.

Adult orbital langerhans cell histiocytosis with frontal bone involvement.

A 28-year-old woman presented with a 2-week history of right upper eyelid swelling and intermittent frontal headaches. CT demonstrated an ill-defined superior right orbital mass with adjacent right frontal bone erosion and undeveloped frontal sinuses. The orbital biopsy revealed tissue strongly positive for CD1a and S100, diagnostic of Langerhans cell histiocytosis.

Orbital compression syndrome in sickle cell disease.

Purpose: To present 3 cases of orbital compression syndrome caused by infarction of the greater wing of the sphenoid in patients with sickle cell disease.

Methods: Case report and review of the literature.

Results: Three patients with sickle cell disease (2 males aged 22 and 16 years, and a 10-year-old girl) who presented with proptosis, limited ocular motility, and chemosis were found to have an infarction of the marrow space of the greater wing of the sphenoid that produced an orbital subperiosteal hemorrhage and exudate demonstrated on MRI.

Isolated lymphoma of the anterior visual pathway diagnosed by optic nerve biopsy.

A 72-year-old previously healthy man developed rapidly progressive visual loss, and brain imaging showed features suggestive of a malignant glioma of the anterior visual pathway. Biopsy of one optic nerve yielded a diagnosis of lymphoma. There was no evidence of an extracranial non-Hodgkin lymphoma, so the conclusion was that this represented a primary central nervous system lymphoma (PCNSL).