Superficial Siderosis Mimicking Amyotrophic Lateral Sclerosis

Driver‐Dunckley, Erika; Hoxworth, Joseph M.; Patel, Naresh P.; Bosch, E. Peter; Goodman, Brent P.

doi:10.1097/cnd.0b013e3181ca3712

Cited by 22 publications

(15 citation statements)

References 22 publications

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“…Paraparesis may be due to iron-related injury in lumbar anterior horns and peripheral nerves, with dysfunction of upper and lower corticospinal neurons leading to atrophy and fasciculations. These features were already observed and described in SS patients [11]. …”

Section: Discussionsupporting

confidence: 69%

Superficial Siderosis and Anticoagulation Therapy: Different Presentations, Different Outcomes

Barreto

Ruano

Cruz

et al. 2012

Case Reports in Neurological Medicine

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Superficial siderosis is a potentially manageable neurodegenerative disorder, caused by chronic subarachnoid haemorrhage and iron deposition along the central nervous system surfaces. Association with oral anticoagulant therapy is well known, but its definite role as a causative agent is yet to be clarified. Two Caucasian women, both under long-term oral anticoagulation: a 74 year old woman with slowly progressive hearing loss and mild cerebellar ataxia; a 72 year old woman suffering from behavioural changes, rapidly progressive cognitive decline and latter developing paraparesis. Magnetic resonance imaging showed striking hypointensities along the surfaces of cerebellum, brainstem, frontotemporal cortices, spinal cord, and lumbar arachnoid therefore suggesting superficial siderosis. No specific bleeding source was found in any of the patients. Anticoagulation could not be stopped in the first patient due to a mechanic valve and slowly progressive worsening occurred. In contrast, for the second patient anticoagulation withdrawal was feasible and marked motor and cognitive improvement ensued. Superficial siderosis is associated with unvarying progression, mostly when no direct source of bleeding is identified. Nonetheless, we verified striking motor and cognitive improvement after anticoagulants withdrawal in one of the patients. This may reinforce the need to consider such modifiable factor in future patient management.

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Section: Discussionsupporting

confidence: 69%

Superficial Siderosis and Anticoagulation Therapy: Different Presentations, Different Outcomes

Barreto

Ruano

Cruz

et al. 2012

Case Reports in Neurological Medicine

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“…There are reports of superficial siderosis mimicking ALS due to deposition of hemosiderin in the ventral nerve roots. 12 In this patient, however, the clinical course had been more rapid than expected for superficial siderosis, and she did not have clinical evidence of deafness or vestibulopathy. Further neuroimaging, including susceptibility weighted MRI studies, would be useful to demonstrate hemosiderin deposition along the surface of the cerebellum, spinal cord, cisternal portion of the cranial nerves (especially cranial nerve VIII), and spinal roots.…”

Section: Clinical Discussionmentioning

confidence: 70%

Ataxia and Weakness in a Young Woman

Beh¹,

Nations²,

Pascual³

et al. 2012

Arch Neurol

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28-year-old woman presented with slowly progressive ataxia and signs of upper and lower motor neuron dysfunction. She had no family history of neurologic disease. Neuroimaging study results were unremarkable, and the diagnosis remained obscure for several years. The differential diagnosis of motor system degeneration in a young adult is broad but focuses on inherited disorders that may have late onset. In this case, close analysis of the clinical features in the history and neurologic examination and interpretation of the laboratory study results led to the diagnosis.

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“…10,14,16,17 These typical ventral spinal CSF collections also have been described in other patients with superficial siderosis syndrome who did not carry a diagnosis of spontaneous intracranial hypotension. 4,11,15,19,20,27 However, it appears that in some of these patients in whom superficial siderosis syndrome was diagnosed, only the cerebellum was involved. 14,16 The source of cerebellar bleeding in our patient population has not been established.…”

Section: Discussionmentioning

confidence: 99%

Chronic cerebellar hemorrhage in spontaneous intracranial hypotension: association with ventral spinal cerebrospinal fluid leaks

Schievink¹,

Maya

Nuño³

2011

SPI

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Object Spontaneous intracranial hypotension is an important cause of new-onset daily persistent headache. Cerebellar hemorrhage has been identified as a possible feature of spontaneous intracranial hypotension. The authors reviewed the MR imaging studies from a group of patients with spontaneous intracranial hypotension to assess the presence of cerebellar hemorrhage. Methods Medical records and radiological images were reviewed in 262 cases involving patients with spontaneous intracranial hypotension who had undergone MR imaging of the brain as well as spinal imaging. Results Chronic cerebellar hemorrhages were found in 7 (2.7%) of the 262 patients with spontaneous intracranial hypotension. These hemorrhages were found in 7 (19.4%) of the 36 patients with a ventral spinal CSF leak and in none of the 226 patients who did not have such a CSF leak (p < 0.0001). The degree of hemosiderin deposits was variable, ranging from mild involvement of the cerebellar folia to widespread superficial siderosis. Only the 1 patient with superficial siderosis had symptoms due to the hemorrhages. The time period between the onset of symptoms due to spontaneous intracranial hypotension and MR imaging examination was significantly longer in those patients with cerebellar hemorrhage than in those with a ventral spinal CSF leak and no evidence for cerebellar hemorrhage (mean 19.6 years vs 2.3 months, p < 0.0001). Conclusions Chronic cerebellar hemorrhage should be included among the manifestations of spontaneous intracranial hypotension. The severity is variable, but the hemorrhage generally is asymptomatic. The underlying spinal CSF leak is ventral and mostly of long duration.

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Superficial Siderosis Mimicking Amyotrophic Lateral Sclerosis

Cited by 22 publications

References 22 publications

Superficial Siderosis and Anticoagulation Therapy: Different Presentations, Different Outcomes

Superficial Siderosis and Anticoagulation Therapy: Different Presentations, Different Outcomes

Ataxia and Weakness in a Young Woman

Chronic cerebellar hemorrhage in spontaneous intracranial hypotension: association with ventral spinal cerebrospinal fluid leaks

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