Ataxia

Overview

Ataxia is considered a symptom of your superficial siderosis medical condition and differs from Hereditary Ataxia or Sporadic Ataxia, which are distinct neurological diseases. Superficial siderosis patients frequently experience many different cerebellar ataxia symptoms affecting, balance, coordination, speech, and swallowing combined with signs of central and peripheral vestibulopathy.¹ Many of the individual symptoms experienced by people may be all be grouped into the ataxia category.

Superficial Siderosis Ataxia Symptoms

Balance, Mobilty, and Coordination

Problems experienced with balance and coordination are often one of the first signs of superficial siderosis related ataxia. Nerve dysfunction may cause a longer and more extended response time between your cerebellum and your muscles. As your muscle control becomes impaired, most individuals will need to resort to a wide-based gait movement in an attempt to stop themselves from falling.

Ataxia was referred to as The Drunken Sailor Syndrome a century ago

The constant battle to control your arm and leg movement is considered a prime contributor to the extreme fatigue felt by many superficial siderosis patients. Impaired voluntary muscle control in the face may cause speech, chewing, or swallowing problems.

Ataxia, Sleep Apnea, and Restless Leg Syndrome

Published research reports and case studies have already linked sleep disorders to cerebellar ataxia. There has been a lot of discussion in our patient community concerning the frequency of Restless Leg Syndrome (RLS) that affects superficial siderosis patients. RLS may be related to one or both identified causes.

If an SS patient is undergoing chelation there is always a danger they are slightly anemic. Iron deficiency has been directly linked to RLS. Additionally, evidence has found a connection between primary sleep disorders related to cerebellar ataxia, focusing on REM sleep behavior disorder (RBD), restless legs syndrome (RLS), periodic leg movement in sleep (PLMS), excessive daytime sleepiness (EDS), insomnia, and sleep apnea.

Management

While there is no cure for neurodegenerative based ataxia, your neurologist should evaluate your symptoms and make referrals to the appropriate specialties to begin a plan of care.

  • Movement Disorder Clinics
  • Physical Therapy
  • Occupatinal Therapy
  • Speech and Language Specialist
  • Neuro-ophthalmologist
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

¹ Bilateral Vestibulopathy in Superficial Siderosis

²Sleep Disorders In Cerebellar Ataxia

Living With SuperficialSiderosis Website PubMed Reference Library 
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