Evoked potential findings in Behçet's disease. Brain-stem auditory, visual, and somatosensory evoked potentials in 44 patients

ABSTRACT:Background:The central nervous system is often involved in Behcet's disease. Most common are meningoencephalitic and brain stem syndromes. Although basal ganglia involvement is not an uncommon finding on necropsy, there are only single reports on extrapyramidal syndromes-dyskinesia, chorea and Parkinsonism in patients with Behcet's disease.Case study:We report a patient fulfilling the criteria of the International Study Group for Behcet's disease. He had recurrent oral ulcerations, bilateral posterior uveitis and retinal vasculitis, skin papules and pustules, and recurrent monoarthritis. Neurologic examination revealed pseudobulbar palsy, slight and asymmetric bilateral pyramidal syndrome, muscle rigidity involving the four limbs, bradykinesia, masked face, and impaired postural reflexes. There was postural tremor in the extremities and myoclonic jerks involving the tongue and face muscles. Magnetic resonance imaging demonstrated small bilateral multifocal hyperintense lesions, with right predilection, involving the periventricular white matter, brain stem and basal ganglia.Conclusions:The Parkinsonian syndrome found in our patient might be due to involvement of both substantia nigra and basal ganglia. This case further emphasizes the wide spectrum of the neurological manifestations of Behcet's disease

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Inflammatory optic neuropathy in Behçet’s disease

Reumatismo ◽

10.4081/reumatismo.2015.835 ◽

2016 ◽

Vol 67 (4) ◽

pp. 156 ◽

Cited By ~ 6

Author(s):

M. S. Khanfir ◽

N. Belfeki ◽

F. Said ◽

T. Ben Salem ◽

I. Ben Ghorbel ◽

...

Keyword(s):

Visual Field ◽

Evoked Potentials ◽

Behçet’S Disease ◽

Visual Evoked Potentials ◽

Optic Neuropathy ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Clinical Manifestations ◽

Behcet's Disease ◽

Visual Evoked

Inflammatory optic neuropathy (ON) is a rare event in Behçet’s disease (BD). We report herein a series of ten BD Tunisian patients with ON and describe its clinical features among them. A retrospective review of BD patients (International Study Group for BD criteria) was performed. The patients were divided into two groups: those presenting an inflammatory ON, and those none. The diagnosis of inflammatory ON was based on the clinical examination, visual field and visual evoked potentials. We analyzed the characteristics of the two groups. Ten patients (2.3%) presented an inflammatory ON among our 440 patients. Inflammatory ON was inaugural in 8 cases. Clinical manifestations were as follows: blurred vision (7 cases) and periorbital pain (3 cases). In two cases, the patients did not complain from ophthalmological symptoms. The fundus revealed a papilledema (2 cases), papillary pallor (4 cases), and was normal in 5 cases. Visual field realized in only three patients showed a scotoma in all cases. Visual evoked potentials revealed increased latency in all cases. All patients received corticosteroids associated to an immunosuppressive agent. The comparative study between the two groups revealed that inflammatory ON was significantly more associated to neurological involvement (p<0.0001) and that the disease was more severe in the ON group (p<0.0001). Inflammatory ON in BD is rare and may occur at an early stage of the clinical course of the disease. Its prevalence is certainly underestimated. A systematic visual evoked potential may be interesting as a screening tool.

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