Spinocerebellar ataxia 4

Overview

Spinocerebellar ataxia 4: An inherited disorder where degeneration of certain parts of the brain and spinal cord results in symptoms such as ataxia, sensory neuropathy and spastic paraplegia.

Symptoms

Spinocerebellar ataxia Lack of control of voluntary leg movements Spinal cord abnormalities Absence of reflexes Gait disturbance

Causes

Cerebellar abscess. Cerebellar abscess commonly causes limb ataxia on the same side as the lesion as well as gait and truncal ataxia. Typically, the initial symptom is a headache localized behind the ear or in the occipital region, followed by oculomotor palsy, fever, vomiting, an altered LOC, and coma. Cerebellar hemorrhage. With cerebellar hemorrhage, a life-threatening disorder, ataxia is usually acute but transient. Unilateral or bilateral ataxia affects the trunk, gait, or limbs. The patient initially experiences repeated vomiting, occipital headache, vertigo, oculomotor palsy, dysphagia, and dysarthria. Later signs, such as a decreased LOC or coma, signal impending herniation. Creutzfeldt-Jakob disease. Creutz-feldt-Jakob disease is a rapidly progressive dementia accompanied by neurologic signs and symptoms, such as myoclonic jerking, ataxia, aphasia, visual disturbances, and paralysis. It generally affects adults ages 40 to 65. Diabetic neuropathy. Peripheral nerve damage due to diabetes mellitus may cause sensory ataxia, extremity pain, slight leg weakness, skin changes, and bowel and bladder dysfunction. Diphtheria. Within 4 to 8 weeks of the onset of symptoms, a life-threatening neuropathy can produce sensory ataxia. Diphtheria can be accompanied by fever, paresthesia, and paralysis of the limbs and, sometimes, the respiratory muscles. Encephalomyelitis. Encephalomyelitis a complication of measles, smallpox, chickenpox, or rubella or of rabies or smallpox vaccination that may damage cerebrospinal white matter. Rarely, it’s accompanied by cerebellar ataxia. Other signs and symptoms include headache, fever, vomiting, an altered LOC, paralysis, seizures, oculomotor palsy, and pupillary changes.