Gait

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Gait ataxia, also referred to as wide-based gait is one of the most common symptoms of Superficial Siderosis. Balance issues are also an element of this category of cerebellar ataxia.

Three sensory groups provide input to the cerebellum to maintain truncal stability. These are the vision, proprioception, and vestibular sense. While standing upright, the Superficial Siderosis patient often is unable to hold still no matter if their eyes are open or closed. Their body may lurch back and forth and from side to side. Patients are not able to walk from heel to toe, in a straight line or up on their tip-toes. They will exhibit poor balance and are unable to stand on one leg.

An unsteady, uncoordinated walk, with a wide base, feet spread out, coming down first on the heel and then on to the toes is considered the classic signs of gait ataxia. This gait is often compared to that of the “drunken sailor.”

Walking with a cane is recommended for safety in the earlier stages. Double hiking poles are excellent when walking over uneven terrain. Use of an aluminum walker will be a benefit when your balance issues go beyond the use of a cane.

Note: The amino acid, acetyl-dl-leucine, has shown benefits for degenerative cerebellar ataxia. In a clinical trial, published in the October 2013 issue of the Journal of Neurology, participants were administered 5 grams of acetyl-dl-leucine per day for 1 week. Researchers reported an average of 20 percent decrease in scores on an ataxia rating scale. Gait coordination and speed, speech, and hand and finger dexterity all showed improvement. No adverse side effects occurred, implying a good risk-to-benefit profile for acetyl-dl-leucine.

 

The use of a specially fitted weighted vest, designed by a physical therapist, has been proven to help patients maintain their balance and re-learn to walk without assistance.

 

Effects of acetyl-DL-leucine in patients with cerebellar ataxia     

 

 

Sources: Superficial siderosis is a rare neurologic disease characterized by progressive sensorineural hearing loss, cerebellar ataxia, pyramidal signs, and neuroimaging findings revealing hemosiderin deposits in the spinal and cranial leptomeninges and subpial layer. The disease progresses slowly, and patients may present with mild cognitive impairment, nystagmus, dysmetria, spasticity, dysdiadochokinesia, dysarthria, hyperreflexia, and Babinski signs. Additional features reported include dementia, urinary incontinence, anosmia, ageusia, and anisocoria. Superficial siderosis MedGen UID: 831707 •Concept ID: CN226971 •Finding Orphanet: ORPHA247245
Living With SuperficialSiderosis Website PubMed Reference Library 

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