BEE Syndromes - Non-inflammatory Causes



Immune-mediated conditions affecting the Brain, Eye, and Ear



Visual or auditory symptoms in conjunction with symptoms attributable to central nervous system (CNS) involvement can be seen in several common and less common diseases. Prompt recognition of an underlying autoimmune basis to these conditions is important, as early diagnosis and treatment can prevent permanent disability.



Non-inflammatory causes:

• Infectious

	• Syphilis

	• Tuberculosis with pachymeningitis

	• HIV

	• Enteroviral infections

	• Herpes virus infections

	• Lyme disease



• Malignant

	• Lymphoma

	• Infiltrating base of skull neoplasms (e.g., nasal squamous cell carcinoma)

	• Carcinomatous meningitis

• Genetic

	• Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations due to three prime repair exonuclease 1 (TREX1) mutations

	• Autosomal dominant optic neuropathy and sensorineural hearing loss—wolfram (WFS1), optic atrophy-1 gene (OPA1) gene

	• Autosomal recessive—Usher syndrome

	• X-linked inheritance—Mohr-Tranebjaerg syndrome

• Mitochondrial

	• Harding’s syndrome—Leber’s hereditary optic neuropathy (LHON) plus MRI lesions and clinical consistent with MS

• Other

	• Syndromes of raised intracranial pressure for example, IIH

	• Migraine with aura



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