Sleep disorders in cerebellar ataxia

Information Download

José L. Pedroso et al, Department of Neurology and Neurosurgery, Universidade Federal de São Paulo, São Paulo SP, Brazil. Arq Neuropsiquiatr 2011;69(2-A):253-257 Cerebellar ataxias comprise a wide range of etiologies leading to central nervous system-related motor and non-motor symptoms. Recently, a large body of evidence has demonstrated a high frequency of non-motor manifestations in cerebellar ataxias, especially in autosomal dominant spinocerebellar ataxias (SCA). Among these nonmotor dysfunctions, sleep disorders have been recognized, although still under or even misdiagnosed. In this review, we highlight the main sleep disorders related to cerebellar ataxias focusing on REM sleep behavior disorder (RBD), restless legs syndrome (RLS), periodic limb movement in sleep (PLMS), excessive daytime sleepiness (EDS), insomnia and sleep apnea.

read more….

Focused Neuro-Otological Review of Superficial Siderosis of the Central Nervous System

Information Download

iSS can be associated with significant neurotologic and cerebellar morbidity; the recurrent SAH variant is frequently clinically symptomatic, has a shorter latency and greater neurotologic disability. In these cases, a thorough search and management of a covert source of bleeding may stop clinical progression. The frequency and clinical course of radiographic iSS after traumatic and post-aneurysmal SAH is largely unknown. Detection of radiographic iSS after trauma or aneurysm bleeding suggests that the slower clinical course could benefit from an effective intervention if it became available. The use of cochlear implants is a valid alternative with advanced hearing impairment.

read more….

A Comprehensive Neuropsychological Assessment of a Case of Superficial Siderosis

Information Download

Superficial siderosis (SS) is a neurodegenerative condition due to the long-term effects of hemosiderin deposition on the sur- face of the brain, cerebellum, brainstem, and spinal cord. SS symptoms include sensorineural hearing loss, ataxia and upper motor neuron signs. SS was diagnostically evasive until magnetic resonance imaging (MRI) became available. As the detection of SS improved, case stud- ies have become more prevalent. To our knowledge, however, this is the first report of SS detailing a comprehensive neuropsychological assessment.

read more….

Optic Neuropathy in Superficial Intracranial Siderosis

Optic Neuropathy in Superficial Intracranial Siderosis Sally L. Painter, MB, BChir, MA, Liberty Mathew, MB, ChB, MRCP, Gerardine Quaghebeur, FRCR, Margaret M. Esiri, DM, FRCPath, John S. Elston, MD, FRCOphth Superficial intracranial siderosis is a degenera- tive condition secondary to recurrent occult subarachnoid hemorrhage. Progressive sensorineural deafness, cere- bellar ataxia, and pyramidal signs are well-documented clinical manifestations, but optic neuropathy is not a recognized feature. We describe 2 patients with clinical and electrophysiological evidence of optic nerve/chiasm dysfunction and MRI signal abnormalities consistent with hemosiderin staining of the anterior visual pathway. In a third case, neuropathological examination of the optic chiasm showed demyelination attributed to hemosiderin deposition. We suggest that anterior visual pathway damage may be underrecognized in this condition.

read more….

Effects of Acetyl-DL-Leucine on Cerebellar Ataxia (Study Protocol)

Cranial Nerves

Effects of acetyl-DL-leucine on cerebellar ataxia (ALCAT trial): study protocol for a multicenter, multinational, randomized, double-blind, placebo-controlled, crossover phase III trial Katharina Feil1,2,11* , Christine Adrion3, Julian Teufel1,2, Sylvia Bösch8, Jens Claassen5, Ilaria Giordano7, Holger Hengel6, Heike Jacobi7, Thomas Klockgether7, Thomas Klopstock1,4,12, Wolfgang Nachbauer8, Ludger Schöls6, Claudia Stendel1,4, Ellen Uslar5, Bart van de Warrenburg9, Ingrid Berger2, Ivonne Naumann2, Otmar Bayer2, Hans-Helge Müller10, Ulrich Mansmann3 and Michael Strupp1,2 Background: Cerebellar ataxia (CA) is a frequent and often disabling condition that impairs motor functioning and impacts on quality of life (QoL). No medication has yet been proven effective for the symptomatic or even causative treatment of hereditary or non-hereditary, non-acquired CA. So far, the only treatment recommendation is physiotherapy. Therefore, new therapeutic options are needed. Based on three observational studies, the primary objective of the acetyl-DL-leucine on ataxia (ALCAT) trial is to examine the efficacy and tolerability of a symptomatic therapy with

read more….

Bilateral Vestibulopathy in Supercial Siderosis

Bilateral Vestibulopathy in Superficial Siderosis Sang-Yeon Lee1, Dong-Han Lee1, Yun Jung Bae2, Jae-Jin Song1, Ji Soo Kim3* Background: Superficial siderosis (SS) is a rare condition in which hemosiderin, an iron storage complex, is deposited in neural tissues because of recurrent subarachnoid bleeding. Hemosiderin deposition in the vestibulocochlear nerve (CN VIII), brain, spinal cord and peripheral nerve can cause sensorineural hearing loss (SNHL) and postural imbalance, but much remains unknown about the vestibular manifestations of SS. Objectives: To report the clinical course, cochleovestibular status, and patterns of vestibulopathy during follow-up of a relatively large case series, and to discuss the possible pathophysiological mechanism of vestibular deterioration. Methods: Six patients diagnosed with SS by magnetic resonance imaging (MRI) were enrolled. Their medical records and radiological findings were retrospectively reviewed, particularly in terms of progression of the vestibulocochlear manifestations and the radiological characteristics.

read more….

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),

read more….

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

read more….

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.

read more….

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.

read more….

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.

read more….

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.

read more….

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.

read more….

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

read more….

Anti-Inflammatory Properties of Arnica

Arnica montana   Köhler–s Medizinal Pflanzen 015

In traditional medicine, alcoholic preparations from flowers
of Arnica montana and Arnica chamissonis ssp. foliosa are
applied externally to treat hematomas, contusions, sprains,
rheumatic diseases, and superficial inflammations of the skin.
The secondary metabolites that mediate the anti-inflammatory
effects are sesquiterpene lactones of the 10a-methylpseudoguaianolide
type like helenalin, 11a,13-dihydrohelenalin, chamissonolid,
and their ester derivatives (1–3). Several studies have
investigated how these natural compounds exert their antiinflammatory
effect.

read more….