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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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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Cerebellar Dysarthria

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 or speaking slowly. If nerve palsies are also involved you may also have trouble controlling the muscles in your mouth and face.   The cranial nerves involved in the articulation of speech. These include:   • Trigeminal nerve (fifth cranial)  supplies nerve function to the chewing muscles • Facial…

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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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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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Dysdiadochokinesia (DDK)

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 nose and then, using the same finger and as quickly as possible, touch the outstretched finger of the person doing the test. Heel-Shin Test You’ll place one heel on one shin just below the knee, and then slide the heel down the shin to the foot. You should aim for…

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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 the total exhaustion so common among Superficial Siderosis is the combination of prescription medications so many patients face every day. Updated: December 15, 2017 ¹Innov Clin Neurosci. 2011 Oct; 8(10): 40–43. Published online 2011 Oct. PMCID: PMC3225130 PMID: 22132370 Fatigue as a Residual Symptom of Depression Steven D. Targum, MD…

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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 compared to that of the “drunken sailor.” Walking with a cane is recommended for safety in the earlier stages. Double hiking poles are excellent when walking over uneven terrain. Use of an aluminum walker will be a benefit when your balance issues go beyond the use of a cane. Note:…

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Hyperreflexia

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 com história progressiva de disfunção neurológica causada por SS-SNC. Os achados clínicos mais frequentes destes pacientes foram ataxia progressiva da marcha, perda auditiva, hiperreflexia e disfunção cognitiva. O diagnóstico de SS-SNC foi firmado de sete meses a 30 anos após o início da doença. Conclusão SS-SNC é uma condição rara…

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Mild Cognitive Impairment

cog test

Mild Cognitive Impairment is when a person experiences a slight but noticeable and measurable decline in cognitive abilities, including memory and thinking skills. These changes are noticeable to the person affected and to family members and friends but will not affect your ability to carry out everyday activities. Depression may amplify cognitive problems. SS patients who have a diagnosis of cerebellar atrophy may experience episodes of impaired recall of new facts, difficulty with planning, organizing and completing new tasks. Initiating a new task and keeping their focus on the task. Cognitive function is often 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 addresses orientation, attention, and memory, with the second addressing verbal and written skills. An overall score between zero and 30 is possible. A score of 27- 30 is indicative of normal cognitive function. A score of 21-26 is considered Mild Cognitive Impairment. A score between 1-20 indicates a diagnosis of dementia. Patients with cerebellar atrophy may have impaired recall of newly learned information or difficulty with “executive functions” such as making plans and keeping…

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