Superficial Siderosis linked ataxia describes the incoordination resulting from your loss of voluntary muscle control, often beginning with your arms or legs. This form of ataxia is considered a symptom of your medical condition and differs from Hereditary Ataxia or Sporadic Ataxia, which are distinct neurological diseases. Superficial Siderosis patients frequently experience different cerebellar ataxia symptoms combined with signs of central and peripheral vestibulopathy.¹
Superficial Siderosis ataxia problems:
- Wide-based gait balance
- Hand, arm, and leg coordination problems
- Fine motor skill affecting hand and finger use
- Slurred or indistinct speech
- Difficulty eating or swallowing
- Unusual eye movements
- Lowered blood pressure upon standing up
- Sexual Dysfunction
- Sleep Apnea²
- Restless Leg Syndrome²
Balance and coordination issues are often an early sign. Nerve dysfunction may cause a longer and more extended response time between your brain 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.
There has been past research published linking sleep disorders to cerebellar ataxia. There has been a lot of discussion in our patient community concerning the frequency of Restless Leg Syndrome (RLS) affecting community members. The evidence has found the primary sleep disorders related to cerebellar ataxias 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.
While there is no cure for neurodegenerative ataxia, specialized physical therapy has proven extremely helpful with balance and wide-based gait issues in some patients. Physicians recommend adding adaptive devices into your daily life; a cane, dual walking sticks, or rolling walker will help guard against dangerous falls. Installing ramps in place of stairs and safety bars in bathrooms will reduce trip hazards. Prescription medications are available to help relieve some of the effects, and the regular use of an APAP or CPAP machine will help control sleep apnea.
Updated: November 15, 2020
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