Superficial Siderosis of the Central Nervous System Secondary to Chronic Bleeding From a Lumbar Paraganglioma

Case Study involving dementia A 73‐year‐old Caucasian male presented to the Hamilton General Hospital Emergency Room with confusion, slurred speech, lower extremity weakness and gait ataxia. According to the patient’s family, his symptoms began five years prior to this episode, when they noticed behavioral changes. He became aloof and introverted. He also complained of ‘ringing’ in his ears. An auditory examination at that time demonstrated hearing loss, for which he started wearing hearing aids. Subsequently, there was a progressive decline in his functional mobility and other activities of daily living. Upon physical examination, the patient was uncooperative. He had a short attention span with brief moments where he demonstrated mental competence. A Folstein mini mental status exam revealed baseline cognitive decline most likely due to his dementia (13/30). The cranial nerves examination demonstrated bilateral hearing loss. He had lower extremity weakness (more pronounced on the right than on the left), hyper‐reflexia and Babinski sign bilaterally. No sensory deficits were noted. Disdiadokokinesia, finger‐nose and heel‐shin dysmetria wer

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Ferriprox® (deferiprone) Prescribing Information

HIGHLIGHTS OF PRESCRIBING INFORMATION These highlights do not include all the information needed to use FERRIPROX safely and effectively. See full prescribing information for FERRIPROX. FERRIPROX® (deferiprone) tablets, for oral use Initial U.S. Approval: 2011 WARNING: AGRANULOCYTOSIS/NEUTROPENIA See full prescribing information for complete boxed warning. INDICATIONS AND USAGE FERRIPROX® (deferiprone) is an iron chelator indicated for the treatment of patients with transfusional iron overload due to thalassemia syndromes when current chelation therapy is inadequate. (1) Approval is based on a reduction in serum ferritin levels. There are no controlled trials demonstrating a direct treatment benefit, such as improvement in disease-related symptoms, functioning, or increased survival. (1) Limitation of Use • Safety and effectiveness have not been established for the treatment of transfusional iron overload in patients with other chronic anemias. (1) DOSAGE AND ADMINISTRATION • 25 mg/kg to 33 mg/kg body weight, orally, three times per day, for a total daily dose of 75 mg/kg to 99 mg/kg body weight. (2) DOSAGE FORMS AND STRENGTHS • 500 mg film-coated tablets with a functional score. (3) CONTRAINDICATIONS • Hypersensitivity to deferiprone or to any of the excipients in the formulation. (4) WARNINGS AND PRECAUTIONS • If infection occurs while on Ferriprox,…

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Ferriprox® (deferiprone) Prescribing Information and Medication Guide

Read this Medication Guide before you start taking FERRIPROX and each time you get a refill. There may be new information. This information does not take the place of talking to your healthcare provider about your medical condition or your treatment. What is the most important information I should know about FERRIPROX? FERRIPROX can cause serious side effects, including a very low white blood cell count in your blood. One type of white blood cell that is important for fighting infections is called a neutrophil. If your neutrophil count is low (neutropenia), you may be at risk of developing a serious infection that can lead to death. Neutropenia is common with FERRIPROX and can become severe in some people. Severe neutropenia is known as agranulocytosis. If you develop agranulocytosis, you will be at risk of developing serious infections that can lead to death.

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Idiopathic infratentorial superficial siderosis of the central nervous system

The superficial siderosis (SS) of the central nervous system (CNS) is a rare condition characterized by a wide range of neurological manifestations directly linked to an acquired iron-mediated neurodegeneration. First described more than 100 years ago, only recently SS has been divided into two distinct entities, according to the distribution of iron deposition in the CNS: cortical superficial siderosis (cSS) and infratentorial superficial siderosis (iSS). Here we describe an adult case of iSS, with detailed clinical and radiological features. Moreover, we extensively review the literature of SS, particularly focusing on the pathogenesis, clinical-radiological classification, diagnostic algorithm and treatment options of this rare condition.

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Vestibular function in superficial siderosis

Vestibular deficits due to SS have rarely been reported
in the otolaryngological literature because early reports
noted the selective deposition of hemosiderin around
the CNS and/or the 8th nerve in contact with the
cerebrospinal fluid, most notably the cerebellum, brainstem,
lining of the ventricles, and spinal cord [1,4].
These deposits around CNS structures and/or the 8th
nerve were considered to be the changes most responsible
for the disequilibrium of SS.

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Effects of acetyl-DL-leucine in patients with cerebellar ataxia

No existing medication has yet been shown to
convincingly improve cerebellar ataxia. Therefore, the
identification of new drugs for its symptomatic treatment is
desirable. The objective of this case series was to evaluate
the efficacy of treatment of cerebellar ataxia with the
amino acid acetyl-DL-leucine (Tanganil). Thirteen patients
(eight males, median age 51 years) with degenerative
cerebellar ataxia of different etiologies (SCA1/2, ADCA,
AOA, SAOA) were treated with acetyl-DL-leucine (5 g/
day) without titration for 1 week.

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Cognitive and social impairments in patients with superficial siderosis

Superficial siderosis of the CNS is a rare condition, caused
by deposition of haemosiderin in the superficial layers
of the CNS due to repeated chronic subarachnoid or intraventricular
hemorrhage. Typically, the hindbrain structures,
especially the cerebellum, are most affected. There
is a surprising lack of studies investigating in detail the
behavioral functioning of patients with such a condition.
In this study, we document for the first time the cognitive,
social and emotional processing of six patients with a
confirmed clinical diagnosis of superficial siderosis.

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Cochlear Implant Patients with Superficial Siderosis Mayo Clinic

It is essential that the CI audiologist not only be aware of the disorder but also be well
versed in the resulting implications for the cochlear implant process. A more thorough case history, an
expanded candidacy test battery, and knowledge of the typical presentation of SSCN are critical. The
diagnosis of SSCN will impact expectations for success with the cochlear implant, and counseling
should be adjusted accordingly. Mayo Clinic Arizona, Scottsdale; {Mayo Clinic Rochester, Rochester, MN

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