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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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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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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Peripheral Neuropathy

neurons-axons

  Neuropathy describes damage to the peripheral nervous system; motor, sensory, or autonomic nerves which move information from the brain and spinal cord to every other part of the body. Peripheral nerve fibers are classified according to size and correspond with myelination: Large nerve fibers are heavily myelinated A-alpha fibers that control motor strength, and A-beta fibers, which control touch sensation. Medium-sized fibers, known as A-gamma fibers, are also myelinated and carry information to the stretch receptors in your muscles. Small fibers include myelinated A-delta fibers and unmyelinated C fibers, which supply skin (somatic fibers) and involuntary muscles (autonomic fibers). They work together to control pain, sense of temperature, and autonomic function. When people talk about peripheral neuropathy, they most often are referring to small fiber neuropathy. Small fiber neuropathy is a disorder of the peripheral nerves that may affect either small somatic fibers, autonomic fibers, or both. Degeneration will lead to sensory changes and autonomic dysfunction when both types of fibers are involved. The symptoms of small fiber neuropathy typically start with burning feet and numb toes. These symptoms will progress to weakness, numbness, and tingling. This may travel upward at which time your hands and finger will also become involved. When autonomic fibers become affected,…

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Phantosmia

Phantosmia, aka Phantom Smell, is smelling an odor that is not really there. Phantosmia is often the first sign of Superficial Siderosis sense of smell problems. Olfactory receptors in your brain transform the chemical signatures of odors into electrical signals (as opposed to physical nerve signals). An odor molecule binds to the olfactory receptors in the nasal mucosa that line your nostrils. This triggers neurons to send messages to your brain. Your olfactory nerve, aka the first cranial nerve, carries these signals directly to the frontal lobe. Our brain categorizes odors into two categories: good and bad. Good smells activate serotonergic systems to release oxytocin. We feel good, happy, and satisfied. Bad smells activate the dopaminergic system, norepinergic system, and epinergic system. These systems kick in our fight-or-flight reaction. We feel bad, scared and repulsed. This disorder is similar to tinnitus. A large percentage of Superficial Siderosis patients who experience phantosmia progress to anosmia.   Updated: June 16, 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,…

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