Superficial siderosis of the central nervous system: an underestimated cause of hearing loss

1997 ◽  
Vol 111 (1) ◽  
pp. 60-62 ◽  
Author(s):  
M. L. Castelli ◽  
A. Husband

AbstractSuperficial siderosis of the central nervous system (CNS) is a rare disease resulting in the accumulation of haemosiderin in the meninges, the brain surface, the spinal cord and the cranial nerves. The pigment is deposited as a result of chronic bleeding in the subarachnoid space. This produces a clinical picture of deafness, ataxia, cranial nerve deficits and in the latest stages dementia. In some cases the source of bleeding can be identified, whilst in others it can not. Despite its rarity the disease should be considered in the differential diagnosis of sensorineural deafness, particularly as it is a progressive and in some cases curable disease which is easily diagnosed by magnetic resonance imaging (MRI). In this case report the haemosiderin was derived from an ependymoma of the fourth ventricle with extension into the cerebello-pontine angle. The first symptom was a worsening sensorineural hearing loss.

1992 ◽  
Vol 107 (1) ◽  
pp. 69-77 ◽  
Author(s):  
Steven M. Parnes ◽  
Susan A. Weaver

Superficial siderosis of the central nervous system is a rare, often unrecognized condition in which there is a deposition of intracellular and extracellular hemosiderin in the leptomeninges, subpial tissue, spinal cord, and cranial nerves as a result of chronic bleeding in the subarachnoid space. On the basis of our cases and a review of the literature, the majority of patients manifest sensorineural hearing loss in addition to cerebellar dysfunction, pyramidal tract signs, and progressive mental deterioration. Recent imaging techniques—particularly CT and MRI—can define the presence of iron deposition, confirming the diagnosis ante mortem. From post mortem specimens, histologic sections demonstrate severe damage to the eighth nerve, with microglial metabolism of iron. Cases will be presented to illustrate these points.


Molecules ◽  
2020 ◽  
Vol 25 (9) ◽  
pp. 2104 ◽  
Author(s):  
Eleonora Ficiarà ◽  
Shoeb Anwar Ansari ◽  
Monica Argenziano ◽  
Luigi Cangemi ◽  
Chiara Monge ◽  
...  

Magnetic Oxygen-Loaded Nanobubbles (MOLNBs), manufactured by adding Superparamagnetic Iron Oxide Nanoparticles (SPIONs) on the surface of polymeric nanobubbles, are investigated as theranostic carriers for delivering oxygen and chemotherapy to brain tumors. Physicochemical and cyto-toxicological properties and in vitro internalization by human brain microvascular endothelial cells as well as the motion of MOLNBs in a static magnetic field were investigated. MOLNBs are safe oxygen-loaded vectors able to overcome the brain membranes and drivable through the Central Nervous System (CNS) to deliver their cargoes to specific sites of interest. In addition, MOLNBs are monitorable either via Magnetic Resonance Imaging (MRI) or Ultrasound (US) sonography. MOLNBs can find application in targeting brain tumors since they can enhance conventional radiotherapy and deliver chemotherapy being driven by ad hoc tailored magnetic fields under MRI and/or US monitoring.


2017 ◽  
Vol 75 (2) ◽  
pp. 92-95 ◽  
Author(s):  
Yara Dadalti Fragoso ◽  
Tarso Adoni ◽  
Joseph Bruno Bidin Brooks ◽  
Sidney Gomes ◽  
Marcus Vinicius Magno Goncalves ◽  
...  

ABSTRACT Superficial siderosis (SS) of the central nervous system (CNS) is a rare and possibly underdiagnosed disorder resulting from chronic or intermittent bleeding into the subarachnoid space, leading to deposition of blood products in the subpial layers of the meninges. Magnetic resonance imaging (MRI) shows a characteristic curvilinear pattern of hypointensity on its blood-sensitive sequences. Methods Series of cases collected from Brazilian centers. Results We studied 13 cases of patients presenting with progressive histories of neurological dysfunction caused by SS-CNS. The most frequent clinical findings in these patients were progressive gait ataxia, hearing loss, hyperreflexia and cognitive dysfunction. The diagnoses of SS-CNS were made seven months to 30 years after the disease onset. Conclusion SS-CNS is a rare disease that may remain undiagnosed for long periods. Awareness of this condition is essential for the clinician.


Author(s):  
Michael J. Aminoff

In 1811, Bell had printed privately a monograph titled Idea of a New Anatomy of the Brain. In it, Bell correctly showed that the anterior but not the posterior roots had motor functions. François Magendie subsequently showed that the anterior roots were motor, and the posterior roots were sensory. This led to a dispute about priority during which Bell republished some of his early work with textual alterations to support his claims. Bell was involved in a similar dispute with Herbert Mayo concerning the separate functions of the fifth (sensory) and seventh (motor) cranial nerves, and Mayo today is a forgotten man. In both instances, Bell deserves credit for the concepts and initial experimental approach, and Magendie and Mayo deserve credit for obtaining and correctly interpreting the definitive experimental findings.


1994 ◽  
Vol 80 (5) ◽  
pp. 931-934 ◽  
Author(s):  
Virginio Bonito ◽  
Cristina Agostinis ◽  
Stefano Ferraresi ◽  
Carlo Alberto Defanti

✓ Superficial siderosis is a rare condition characterized by deposition of hemosiderin in the leptomeninges and in the subpial layers of the brain and spinal cord. It is associated with cerebrospinal fluid abnormalities consistent with recurrent bleeding into the subarachnoid space. The usual symptoms are hearing loss, ataxia, spastic paraparesis, sensory and sphincter deficits, and mental deterioration. A case is presented of severe superficial siderosis of the central nervous system in a 51-year-old man who had suffered a brachial plexus injury at the age of 20 years. The diagnosis was made by means of magnetic resonance imaging 16 years after the initial symptoms, which comprised bilateral hearing loss and anosmia. Subarachnoid bleeding was due to traumatic pseudomeningocele of the brachial plexus, a very unusual cause of superficial siderosis. This case is interesting insofar as the surgical treatment prevented further bleeding and possibly progression of the disease.


2021 ◽  
Vol 10 (11) ◽  
pp. e270101119579
Author(s):  
Cássio Marques Perlin ◽  
Lanusa Alquino Colombo ◽  
Anderson Dillmann Groto ◽  
Bruno Gleizer da Silva Rigon

Superficial Siderosis (SS) of Central Nervous System is a rare disease characterized by the deposit of hemosiderin in the brain and spinal cord. Clinically, it is characterized by progressive sensorineural ataxia and deafness associated with injury of superior motor neuron. The diagnosis is made by magnetic resonance imaging (MRI) of the encephalon and spinal cord. The objective of the study is to report the case of a patient with characteristic elements of the syndrome, accompanied in a private medical clinic.


2004 ◽  
Vol 25 (2) ◽  
pp. 142-149 ◽  
Author(s):  
Dominique Vibert ◽  
Rudolf Häusler ◽  
Karl-Olof Lövblad ◽  
Gerhard Schroth

2015 ◽  
Vol 14 (4) ◽  
pp. 238-241
Author(s):  
Ioana Cociasu ◽  
◽  
Irene Davidescu ◽  
Ioan Buraga ◽  
Bogdan O. Popescu ◽  
...  

The most common tumours of the central nervous system, meningiomas are frequently diagnosed by accident when patients undergo imaging studies of the brain for other reasons. Most patients lack symptoms and thus can live their whole lives without knowing they have a brain tumour. Less fortunate patients seek medical advice for troubling symptoms – like seizures or disturbances of the cranial nerves – get surgery for the excision of the tumour and years later fi nd out their tumour has come back. We are presenting the case of such a patient with a recurrent parietal meningioma.


1990 ◽  
Vol 38 (4) ◽  
pp. 387 ◽  
Author(s):  
GK Snyder ◽  
B Gannon ◽  
RV Baudinette

The vasculature and microvasculature in the central nervous system of the lizard Trnchydosaurus rugosus was examined by means of scanning electron microscopy of vascular corrosion casts. The arterial supply to the brain is from paired internal carotids. Secondary branches from these feeder arteries form a network over the surface of the lizard brain, giving rise to artery-vein vessel pairs. The vessel pairs bend at right angles to the brain surface, giving rise to terminal arteriole-venular pairs which penetrate the brain substance. Capillaries arise at fairly regular intervals along the vessel pairs, each originating from the arteriole and terminating in the venule at a site contiguous with its origin from the arteriole. In this way all of the capillaries in the central nervous system of the lizard form countercurrent loops. Anastomotic connections between arteriole-venular pairs do not occur, each arterial vessel being an end artery.


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