J Korean Neurosurg Soc.  2011 Jul;50(1):68-71. 10.3340/jkns.2011.50.1.68.

Rapid Atypical Progression of Neuro-Behcet's Disease Involving Whole Brainstem and Bilateral Thalami

• Lee SK
• Choi SJ
• Kim SD
• Lim DJ

• KMID: 2066858
• DOI: http://doi.org/10.3340/jkns.2011.50.1.68

Abstract

We present a case of Neuro-Behcet's disease with an unpredictable clinical course. A 47-year-old man was admitted to the neurosurgery department of our hospital with a mild headache. Three days after admission, his consciousness suddenly decreased and respiratory distress progressed rapidly. A brain MRI revealed that the previously observed abnormal signal had extended markedly to both the thalamic areas and the entire brain stem, and the surrounding brain parenchyma were compressed by cerebral edema. Based on the patient's symptoms of recurrent oral and genital ulcers, skin lesions, and uveitis, a rheumatologist made a diagnosis of Behcet's disease with CNS involvement. The patient was treated with high-dose methylprednisolone with respiratory assistance in the intensive care unit for 9 days and his neurologic symptoms improved remarkably. Neuro-Behcet's disease must be considered in the differential diagnosis in rapidly deteriorated young neurological patients along with a stroke, low-grade glioma, multiple sclerosis, and occlusive venous disease.

Keyword

Behcet's disease; Neuro-Behcet's disease; Central Nerve System manifestation; Neurological deterioration

MeSH Terms

Brain
Brain Edema
Brain Stem
Consciousness
Diagnosis, Differential
Glioma
Headache
Humans
Intensive Care Units
Methylprednisolone
Middle Aged
Multiple Sclerosis
Neurologic Manifestations
Neurosurgery
Skin Ulcer
Stroke
Uveitis
Methylprednisolone