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 

read more….

Anosmia

2A730E6F 9EC1 4595 92B3 32C45EB3DA66 scaled

  Anosmia is the complete loss of your sense of smell from damage to the first cranial nerve and parts of the fifth cranial nerve. It is a very common symptom of Superficial Siderosis. The progression starts very slowly. Most people will notice off odors or phantom smells at first. Over time the death of you olfactory receptor neurons lead to the complete loss of your sense of smell. Your sense of smell 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. These basic flavors comes from the taste 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 cold, sweet, soft

read more….

Ataxia

DA8B5F5E EC9C 4C52 82A9 0E2C72F46375

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.

read more….

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 

read more….

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.

read more….

Cerebellar Dysarthria

2A730E6F 9EC1 4595 92B3 32C45EB3DA66 scaled

Ataxic cerebellum dysarthria is a sensorimotor speech disorder associated with damage to the cerebellum or its input and output pathways.¹   The cerebellums role in feed-forward processing has been linked to speech motor control. Neuro imaging studies led researchers Kristie Spencer and Dana Slocomb, Department of Speech and Hearing Sciences, University of Washington, Seattle, to establish two cerebellar cortical regions are responsible for the feedforward motor commands for speech: the anterior paravermal and superior lateral areas. Previously ataxic dysarthria had been classified as a disorder of motor execution, focusing on uncoordinated and hypotonic muscles.² Motor nerve difficulties clearly contribute to ataxic dysarthria but this doesn’t address the processing abnormalities controlled by the cerebellum that take place during the planning phase of speech. Difficulty in the pronunciation of words due to neurologic dysfunction of the nerves that control the facial muscles, tongue, lips, and throat present as a slurring of words

read more….

Craniospinal Hypotension

Craniospinal Hypotension, also known as Intracranial 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. “This was my first true symptom. I could not effectively describe what was happening to doctors. I had double sciatica at the same time, so it was dismissed. I kept it to myself and knew something was seriously wrong. The pain came on in a split second, felt as if, it traveled from the bottom of my spine, shot to my

read more….

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. 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 also a symptom, short-term memory loss in cases of Superficial Siderosis can be traced to stress, depression, and cerebellar difficulties.  Memory loss alone does not mean you have early stage dementia or will ever progress to dementia. It’s estimated only

read more….

Dental Pain, Not feeling

Regular dental care is essential in patients with superficial siderosis. It’s very rare, but there have been a few documented cases of sensory neuropathy impairing the ability to feel dental pain associated with cavities, abscessed teeth or a cracked tooth.   We are currently searching for documentation of this symptom

read more….

Depression

One of the more common symptoms superficial siderosis patients will experience, besides Sensorineural Hearing Loss or Ataxia, is depression. As your clinical symptoms progress patients are forced to adjust to the drastic quality of life changes. Stressors take over challenging you daily. 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 develop in Superficial Siderosis patient when cerebellar degeneration is present.  New studies have shown the cerebellum plays a critical role in mood function.² Depression,

read more….

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

read more….

Dysdiadochokinesia (DDK)

2A730E6F 9EC1 4595 92B3 32C45EB3DA66 scaled

DDK is a symptom brought on by damage to the cerebellum and falls under the ataxia grouping. The signs of Dysdiadochokinesia include changes in balance and gait, slow, awkward or rigid movements. A decline in the coordination of the arms, hands, or legs. Inarticulate or incomprehensible speech patterns and impairment of the ability to stop a directional movement and start the same action in the opposite direction. For example, a person with DDK may have difficulty screwing or unscrewing a light bulb. They may also have a hard time repeating one to three syllables in a row quickly. Your neurologist can perform simple in-office testing to evaluate DDK. Rapidly Alternating Movement Evaluation You’ll hold the palm of one hand on a flat surface (often the upper thigh), and then continuously flip the hand palm side up, then back to palm side down as fast as possible. Point-To-Point Movement Evaluation You’ll be asked to touch your

read more….

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”.

read more….

Fatigue

Superficial Siderosis patients who suffer from cerebellar atrophy induced ataxia will feel excessive fatigue just from going about your normal daily activities. A person suffering from ataxia has to fight their own body to perform regular movements. This impairment of cerebellar regulation of coordinated movement triggers increased fatigue because you are continually forced to exert more effort. Your gait is compromised, so it takes all your concentration to keep your balance and try to walk in a reasonably straight path. You spend your whole day trying to make your body comply; you consciously have to exert muscle and coordination control, so you feel real physical and mental fatigue. Depression is one of the most common symptoms of Superficial Siderosis patients. The connection between depression and physical fatigue is well documented. It can become an unintended cycle, constant exhaustion causes a deeper depression¹. Deep depression exacerbates fatigue. One more contributing factor to

read more….

Gait

Gait ataxia, also referred to as wide-based gait is one of the most common symptoms of Superficial Siderosis. Balance issues are also an element of this category of cerebellar ataxia. Three sensory groups provide input to the cerebellum to maintain truncal stability. These are the vision, proprioception, and vestibular sense. While standing upright, the Superficial Siderosis patient often is unable to hold still no matter if their eyes are open or closed. Their body may lurch back and forth and from side to side. Patients are not able to walk from heel to toe, in a straight line or up on their tip-toes. They will exhibit poor balance and are unable to stand on one leg. An unsteady, uncoordinated walk, with a wide base, feet spread out, coming down first on the heel and then on to the toes is considered the classic signs of gait ataxia. This gait is often

read more….

Headache

2A730E6F 9EC1 4595 92B3 32C45EB3DA66 scaled

It’s been challenging to research the exact cause of Superficial Siderosis associated headache. Everyone has experience with headache pain: tension, stress, dehydration or eye strain. Take a couple of ibuprofen or acetaminophen and go on about your business. The headaches linked to Superficial Siderosis can cause excruciating pain. Dr. Levy has hypothesized there is a connection between the onset of a headache and when you are experiencing an active bleed. Fresh blood infiltrates your central nervous system, irritating surface linings and causes inflammation. Your pain and stiffness in your neck area is the body’s response to the swelling. The following is the closest explanation we could find for a migraine caused by leaking blood. Hopefully, this is a close estimation of what is happening: “….The stimulation of the trigeminal nerve causes blood vessels in the coverings of the brain to dilate, and this allows white blood cells to leak into

read more….

Hyperreflexia

2A730E6F 9EC1 4595 92B3 32C45EB3DA66 scaled

Hyperreflexia is a sign of upper motor neuron damage and is associated with spasticity. The most common signs are finger flexion reflexes, jaw jerks, involuntary rhythmic muscular contraction and relaxation, and reflexes where an impulse enters one segment and activate motor neurons located in many other segments (Irradiation of Reflexes). Detrusor hyperreflexia affects the contractile mechanism of the bladder. Overactive detrusor hyperreflexia is responsible for bladder incontinence. Dyssynergia keeps your bladder from relaxing so it will not empty completely.   Reference: Arq. Neuro-Psiquiatr. vol.75 no.2 São Paulo Feb. 2017 http://dx.doi.org/10.1590/0004-282×20170001 RESUMO Siderose superficial (SS) do sistema nervoso central (SNC) é uma doença rara e provavelmente subdiagnosticada, resultante de sangramento crônico no espaço subaracnóide, levando ao depósito de produtos sanguíneos nas camadas meníngeas subpiais. Ressonância magnética (RM) mostra um padrão curvilíneo característico com hipointensidade nas suas sequências sensíveis a sangue. Métodos Série de casos coletados de centros brasileiros. Resultados Apresentamos 13 casos de pacientes

read more….

Mild Cognitive Impairment

When a superficial siderosis patient begins to exhibit a slight but noticeable and measurable decline in cognitive abilities, including memory and thinking skills, your physician may diagnose this as Mild Cognitive Impairment.  These changes are most noticeable to family members and friends of the person affected. While these changes are problematic, MCI will not affect your ability to carry out everyday activities. Depression is known to amplify cognitive problems. SS patients previously diagnosed with cerebellar atrophy may experience episodes of impaired recall of new facts, difficulty with planning, organizing, and completing tasks. Initiating a new task and keeping their focus on the task. It often becomes difficult to keep your thoughts in the proper sequence. Cognitive function is evaluated by the administration of either the Mini-Mental State Examination (MMSE) or the Saint Louis University Mental Status Examination (SLUMS). Both tests consist of 11 questions in two sections: the first part

read more….

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

read more….

Myelopathy

Myelopathy is one of the three major symptoms of superficial siderosis and is defined as an acute or subacute spinal cord dysfunction, slowly progressive, and caused by demyelination. Non superficial siderosis related conditions will also contribute negatively to the progression. Myelopathy is aggravated by arthritic changes (spondylosis) which cause narrowing of the spinal canal (spinal stenosis) resulting in compression of the spinal cord. Myelopathy is typically diagnosed by a clinical exam. The signs and symptoms may include: Neck, arm, leg or lower back pain. Tingling, numbness or weakness. Difficulty with fine motor skills, such as writing, using utensils or buttoning a shirt Difficulty walking upper motor neuron signs: weakness, clumsiness, altered tonus hyperreflexia: increased or abnormal twitching or spastic reflexes  lower motor neuron signs: muscle group weakness in the area of spinal cord compromise, muscle atrophy, hyporeflexia, muscle hypotonicity or flaccidity sensory deficits Loss of urinary or bowel control  sexual dysfunction. Myelopathy can

read more….