Roussy Levy hereditary areflexic dystasia

December 31, 2014

Overview

An inherited ataxia (incoordination) involving muscle wasting, kyphoscoliosis and absence of tendon reflexes.

Symptoms

* Walking difficulty * Clumsiness * Hand tremor * Mental deficiency * Muscle wasting in legs * Muscle wasting in hands * Incoordination * Clawfoot * Absence of tendon reflexes * Kyphoscoliosis * Sensory changes * Degeneration of spinal cord radicular areas * Degeneration of spinal cord posterior roots

Causes

* Orthopedic issues affecting the foot, ankle, leg, knee, or hip * Peripheral neuropathy (sensory and/or motor) –Slapping gait: Sensory neuropathies may result in a tendency to slap the feet firmly against the ground to improve proprioceptive input –Steppage gait: Seen in patients with foot drop –The classic tabetic gait combines both stepping and slapping gaits * Mononeuropathy/radiculopathy affecting the lower extremities may result in gait abnormalities (e.g., either a peroneal neuropathy or L5 radiculopathy can cause a unilateral steppage gait) Myelopathy –Patients with bilateral lower extremity weakness and hypertonicity secondary to a spinal cord lesion may exhibit a spastic gait with stiffness of both legs and a tendency toward scissoring of the legs with walking Brainstem or cortical lesions (e.g., multiple sclerosis , CVA) –Most commonly result in a hemiparetic gait with circumduction of the weak leg Cerebellar lesions –Result in an ataxic gait, which tends to be wide-based, irregular, and staggering

Prognosis

The 'prognosis' of Roussy Levy hereditary areflexic dystasia usually refers to the likely outcome of Roussy Levy hereditary areflexic dystasia. The prognosis of Roussy Levy hereditary areflexic dystasia may include the duration of Roussy Levy hereditary areflexic dystasia, chances of complications of Roussy Levy hereditary areflexic dystasia, probable outcomes, prospects for recovery, recovery period for Roussy Levy hereditary areflexic dystasia, survival rates, death rates, and other outcome possibilities in the overall prognosis of Roussy Levy hereditary areflexic dystasia. Naturally, such forecast issues are by their nature unpredictable.