Anisocoria

Anisocoria is when the pupils of both eyes are uneven in size. Defined when the difference is 0.4 mm or greater between the pupils.  It is an indication of demyelination of the optic nerves. 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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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 categories: salty, sweet, bitter, sour, and umami. This flavor comes from the buds located on your tongue. When you lose your sense of smell, you lose the ability to enjoy the nuances of flavor. Eat chocolate ice cream with your eyes closed. A person with anosmia will tell you it’s…

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Ataxia

superficial siderosis ataxia

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 separate neurological diseases. Ataxia is one of the three main clinical symptoms experienced by Superficial Siderosis patients. It is the parent symptom of many related problems including gait and posture, fine motor skill coordination, speech, swallowing, visual abnormalities, fatigue, mild cognitive impairment and mood difficulties. This is a paper on treatment of cerebellar ataxia: Effects of acetyl-DL-leucine in patients with cerebellar ataxia                 Updated: December 15, 2017 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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Body Pain

Chronic body pain, weakness or numbness is the result of neurological induced myelopathy, peripheral neuropathy, and spasticity.           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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Bowel Function

Brainstem and spinal cord involvement along with autonomic peripheral neuropathy may result in alternating episodes of constipation or bowel incontinence. This type of constipation does not respond very well to laxatives or fiber since it reflects a widespread motility problem affecting the whole gut. 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. ”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.”     Updated: December 14, 2017 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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Cerebellar Dysarthria

Cerebellar Dysarthria is a motor speech disorder that causes difficulty in the pronunciation of words due to neurologic disturbances of the nerves that control the facial muscles, tongue, lips, and throat. It often presents as a slurring of words or speaking slowly. You may also have trouble controlling the muscles in your mouth and face. 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 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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Craniospinal Hypotension

Craniospinal Hypotension is not an actual symptom of Superficial Siderosis. It presents as a secondary result of the traumatic event (accident or surgery) which caused your Superficial Siderosis. Craniospinal Hypotension is a condition caused by a dural leak of your cerebral spinal fluid. Your body produces spinal fluid every day, but if there is a leak, then your system 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 craniospinal hypotension are a postural headache, nausea, vomiting, neck pain, visual and hearing disturbances, and vertigo. Diagnosis is dependent on the postural component of a headache; pain increases or eases with positional changes of your head and body. When symptoms are significant, the first choice is an epidural blood patch. In this procedure, some blood is taken from the patient’s arm vein and is injected into the spinal canal in the space outside the dura. Epidural patching sometimes involves the use of fibrin sealant. These procedures may be repeated several times if the improvement is incomplete or does not last. If a blood patch procedure is not effective you may have to have a…

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Dementia

MRI Color Enhanced

  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. 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. Dementia is classified as a group of symptoms affecting memory, thinking and social behavior enough to interfere with daily functioning. Behavioral indications include personality changes, depression, anxiety, inappropriate behavior, paranoia, agitation, hallucinations and decreased verbal fluency. Patients will show a difficulty in finding words, reasoning or problem-solving, handling complex tasks, planning, and organizing, coordination, and motor functions, confusion and disorientation.¹ Even though a symptom is short-term memory loss, memory loss has a different cause. So memory loss alone does not mean you have dementia or will ever progress to dementia. It’s estimated only 25% of Superficial Siderosis patients with cognitive difficulties will progress into clinical dementia.² This case study describes a Superficial Siderosis patient who had progressed into dementia along with other Superficial Siderosis related symptoms. It is important to note beside Superficial Siderosis he had a past medical history of hypertension, cerebellar strokes, left ventricular ejection fraction of…

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Depression

The most common clinical symptom Superficial Siderosis patients suffer besides sensorineural hearing loss and ataxia is depression. As your clinical symptoms progress stressors take over; slowly your quality of life begins to change. Chronic stress can naturally evolve into depression, but there is also evidence of a biological connection. Screening Superficial Siderosis patients for depression needs to be a regular part of their plan of care. Loss of concentration, sleep disturbance, cognitive and behavior changes can be the cause of and caused by depression. Increased physical pain and fatigue can be real consequences in a depressive state. Friends or family often believe feeling sad is run-of-the-mill, so it’s easy to miss the distress.¹ Personality and mood disorders often present in Superficial Siderosis patient when cerebellar degeneration is present.  New studies have shown the cerebellum plays a critical role in mood function.² 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.   Updated: June 19, 2018 Sources: Superficial siderosis is a rare neurologic…

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