Refractory vitamin E deficiency is thought to have irreversible effects on neurologic function. We report an adolescent boy with severe refractory vitamin E deficiency due to progressive familial intrahepatic cholestasis (PFIC) type 2. His consequent neurologic dysfunction included severe ataxia, dysmetria, dysarthria, and cranial nerve VI palsy. He underwent liver transplantation at age 13 due...

Neurologic symptoms are a prominent and often disturbing component of the migraine syndrome in many patients. Collectively termed "aura," migraine-related neurologic symptoms include visual, sensory, language, and motor disturbance. They occur in about one quarter of migraine patients, are classically transient, and are thought to occur as the result of cortical phenomena. Recently, motor sympt...

PURPOSE OF REVIEW Just as gastrointestinal dysfunction may develop in the setting of neurologic disease, neurologic dysfunction may become evident in the setting of gastrointestinal disease. This article describes the range of neurologic features that have been described in three primary gastrointestinal diseases: celiac disease and gluten-related disorders, inflammatory bowel disease, and Whip...

Diseases of the ear often cause signs of neurologic dysfunction because of damage of peripheral nervous system structures associated with the middle and inner ear. Vestibular dysfunction, facial paralysis, Horner's syndrome, and hearing deficits are the most common neurologic deficits that accompany middle and inner ear disease. Differentiating these signs from disease of the central nervous sy...

Introduction: Laryngeal dysfunction may be divided into three categories; organic, neurologic and functional disorders. Dysphonia and hoarseness are the most common symptoms and, in some cases, the only signs of laryngeal dysfunction. In differential diagnosis of any type of chronic hoarseness, a neoplastic process must be considered and, thus continuous light video laryngoscopy can provide imp...