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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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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Diplopia

Diplopia is the medical term for double vision. Binocular double vision occurs in Superficial Siderosis patients suffering from cranial nerve palsies affecting the III, IV and VI nerves. When the images produced by each eye do not match your brain misinterprets the information so you see two misaligned objects. Cranial nerve palsies were the most common cause of binocular diplopia (67%) with half of these being abducent nerve palsies.¹ This type of diplopia disappears when one eye is covered. Wearing an eye patch or prism lens eyeglasses have been found to be helpful in managing diplopia.   ¹To evaluate the causes and outcomes for patients presenting with diplopia to an eye casualty department. Patients presenting with diplopia as a principal symptom, who were referred to the Orthoptic Department from Moorfields Eye Casualty over a 12-month period, were retrospectively investigated. One hundred and seventy-one patients were identified with complete records in 165 cases. There were 99 men and 66 women with an age range of 5-88 years. Monocular diplopia accounted for 19 cases (11.5%), whereas 146 patients (88.5%) had binocular diplopia. Cranial nerve palsies were the most common cause of binocular diplopia accounting for 98 (67%) of cases. Isolated sixth nerve palsy was the…

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Dysphagia

Dysphagia is a difficulty in swallowing, either solids or liquids when there is a problem with the neural control of the swallowing process. Screening assessments for dysphagia are essential as the severity progresses to the determine if there is a danger of aspiration or before malnutrition occurs from an insufficient caloric intake. Signs of Dysphagia include trouble chewing food, solids or liquids accidentally entering into your airway, choking, aspiration pneumonia, a feeling your food is stuck in your throat, regurgitation, and reflux. For an in-depth look at the concerns dysphagia presents for superficial siderosis patients please read our article ”The Danger Of Dysphagia”.

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Motor Skills Impairment

Motor skills are motions carried out when the brain, nervous system, and muscles work in concert. The cerebellum is deeply interconnected with sensory-motor areas of the cerebral cortex. Cerebellar ataxia and motor-based neuropathy damage both contribute to the loss or impairment of motor skills. Cerebellar ataxia affects the fine motor skills, small movements like opening a jar, holding a fork, cutting your food or using a small tool. Fine motor skills use the muscles in your fingers, hands, wrists, toes, lips, and tongue. Motor-based neuropathy affects the gross motor skills, the large muscles in the arms, legs, torso, and feet. These skills are responsible for the bigger movements like sitting, standing, walking, running and balance.     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…

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Myelopathy

Myelopathy covers the areas of the brain stem and spinal cord dysfunction. The spinal cord and nerves control strength, sensation, bladder function, autonomic function. Many different symptoms can result from myelopathy, including weakness, numbness, pain, spasticity, urinary retention, urinary incontinence, constipation or bowel incontinence. Please refer to the individual symptoms that are grouped under the myelopathy category for more specific information.     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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Nerve Palsies

A diagnosis of Nerve Palsy in Superficial Siderosis cases is a blanket category which includes seventh cranial nerve impairment and dysfunction from additional nerves such as the third, fourth and sixth cranial nerves. Double vision (Diplopia) Drooping eyelid(s) Eye positions (outward, inward, upward, downward, crossing) A weakness of facial expression muscles A lost or reduced ability to produce tears or saliva Loss of the ability to sense taste on the front 2/3rds portion of the tongue.   Updated: June 18, 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 Living With SuperficialSiderosis Website PubMed Reference Library 

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Nystagmus

Nystagmus is a vision condition in which the eyes make repeated jerking uncontrollable movements. These movements often result in difficulty reading, focusing and depth perception problems. These uncontrollable and involuntary eye movements can be from side to side (Horizontal), up and down (Vertical) or in a circular (Rotary) motion. Also, movements may also be classified as either Upbeat or Downbeat. Midline cerebellar disturbance is indicated in cases of Superficial Siderosis. Nystagmus is caused by dysfunction of the vestibular nerve, the vestibular nucleus within the brainstem, or parts of the cerebellum that transmit signals to the vestibular nucleus and is classified under ataxia. Nystagmus will affect your vision by changing how you see things because of this constant movement. It will impact your ability to focus, maintain a clear image, watch television or read. Severe nystagmus can negatively affect your ability to drive safely and in some cases progress to the point you may be declared legally blind. Fatigue or stress is known to cause eye movements to increase. A specialized contact lens is considered to offer the best alternative by moving with your eye. In mild cases, the weight of contacts may help slow down eye movement.     Updated: December 15,…

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