Superficial Siderosis associated ataxia describes the incoordination symptoms which come from your loss of voluntary muscle control 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 separate neurological diseases. This is a direct result of the degenerative changes in your central nervous system.
Ataxia is one of the three classic conditions affecting a large percentage of superficial siderosis patients. Community members will often experience different combinations of cerebellar ataxia symptoms along with signs of central and peripheral vestibulopathy.¹
Balance and coordination issues are often the first sign there is a problem. Nerve cell degeneration will cause a longer and longer 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 refered 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.
Superficial siderosis ataxia symptoms:
- 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
There is no cure for neurodegenerative ataxia but physical therapy has proven extremely helpful in reducing the severity of symptoms in some patients. The use of adaptive devices in your daily life is highly recommended. A cane, dual walking sticks, or walkers will help guard against dangerous falls. Installing ramps in place of stairs and safety bars in bathrooms will reduce trip hazards. Medications are available to help relieve some symptoms and the regular use of an APAP or CPAP machine will help control sleep apnea.
This link will download a paper on drug treatment for cerebellar ataxia:
Effects of acetyl-DL-leucine in patients with cerebellar ataxia
Updated: November 11, 2018
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