Anosmia

Anosmia is the loss of the sense of smell caused by damage to the first cranial nerve and parts of the fifth cranial nerve. The first cranial nerve is responsible for qualitative odor sensations like flowers or fresh cut grass. The fifth cranial nerve controls the overtones of smell like warmth, coolness, sharpness, and irritation. An onion or ammonia will not only smell strong but they will also cause a body response. Smell an onion and tears will form. Damage to the parts of the fifth cranial nerve that connects to the tongue is rare. Anosmia will still let you distinguish stinging, burning, cooling and sharpness through the nerve endings in your tongue. Complete loss of taste and smell is called ageusia. Your sense of smell is what controls your concept of the more delicate qualities of taste. When you have anosmia, you can still distinguish the five basic taste…

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Ataxia

superficial siderosis ataxia

Ataxia is a category grouping that includes problems with gait and posture, fine motor skill coordination, speech, swallowing, visual abnormalities, fatigue, cognitive and mood difficulties. Superficial Siderosis associated ataxia is used to describe a symptom of incoordination which is linked to your degenerative changes in the central nervous system. This form of ataxia is a symptom of your medical condition and is different from Hereditary or Sporadic Ataxia which is a separate neurological disease.   Effects of acetyl-DL-leucine in patients with cerebellar ataxia     

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Bowel Function

Brainstem and spinal cord involvement may result in alternating constipation or bowel incontinence. This type of constipation does not respond very well to laxatives since it reflects a widespread motility problem affecting the whole gut. ”This is rare, no familial form of slowly progressive neuropathy affecting some autonomic functions…Patients may have gastrointestinal dysfunction manifest many years before other peripheral sensory symptoms present.” Although rare, this condition suggests that autonomic dysfunction of gut motility is early evidence of sensory and motor neuropathy from peripheral or cranial nerve degeneration.  

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Cerebellar Dysarthria

Cerebellar Dysarthria is a motor speech disorder that causes difficulty in the pronunciation of words due to neurologic disturbances of facial muscles, tongue, lips, and throat. It often presents as a slurring of words or speaking slowly. Several cranial nerves are involved in the articulation of speech. These include: • Trigeminal nerve (fifth cranial) which supplies nerve function to the chewing muscles • Facial nerve (seventh cranial) which provides the facial muscles • Hypoglossal nerve (twelfth cranial) which controls the tongue

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Craniospinal Hypotension

Secondary Craniospinal Hypotension is a condition caused by a dural leak of your cerebral spinal fluid. You produce spinal fluid every day, but if there is a leak, then your body may not be able to replace enough volume to support and cushion your brain. This low volume creates a negative pressure inside your brain cavity. The signs of cranial hypotension are postural headache, nausea, vomiting, neck pain, visual and hearing disturbances, and vertigo. Diagnosis is dependent on the postural component of a headache; pain is increasing or decreasing with positional changes of your head and body. Your dural leaks location will not be identified in some cases but if surgery is successful in finding and closing the leak headaches will stop. Secondary Cranial Hypotension is not a true symptom of Superficial Siderosis. It presents as a secondary result of the traumatic event (accident or surgery) which caused your Superficial…

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Dementia

mri cSS

Dementia is a group of symptoms affecting memory, thinking and social behavior enough to interfere with daily functioning. Even though a symptom is memory loss, memory loss has different causes. So memory loss alone does not mean you have dementia. Dementia is the “progressive decline in cognitive function due to organic damage to the cerebellum and parts of the brain. Areas particularly affected include memory, attention, judgment, language, and problem-solving.” The neuropsychological findings include deterioration of both short term and long term memories of past personal experiences. Memory loss is usually first noticed by a spouse or someone else close. Difficulty with finding words, reasoning or problem-solving, handling complex tasks, planning and organizing, coordination and motor functions, confusion and disorientation. Behavioral and speech include personality changes, depression, anxiety, inappropriate behavior, paranoia, agitation, hallucinations and decreased verbal fluency. It’s estimated 25% of superficial siderosis patients with cognitive difficulties will progress into clinical dementia.

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Depression

Personality and mood disorders often present in superficial siderosis patient when there is cerebellar degeneration  present.  New studies have shown the cerebellum plays a critical role in mood function. Depression, anxiety and irritability symptoms are all intensified. Depression in turn will elevate pain levels, magnify cognitive difficulties and be responsible for a general feeling of melancholy. Your yearly plan-of-care should include screening for depression. Therapy sessions with a psychiatrist or psychologist will help control feelings and mood.

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