Figure 1
Tra T2*GE
A hypointense small margin is visible at the pons and the upper cerebral vermis in comparison to a regular patient.
Cerebral hemosiderosis
SectionNeuroradiology
Case TypeClinical Cases
AuthorsLothar Albrecht, Thomas Labuhn, Michael Baese
Connected authors
68 years, male
Clinical History
A 68 year old male patient with slowly progressive ataxia and dementia received a cranial MRI.
Imaging Findings
A 68 year old male patient suffered from bilateral deafness. Since two years a cerebellar ataxia had been increased, and pyramid signs had become positive. A weakness of memory and a lack of concentration indicated dementia.
A cerebral MRI was performed with a 1.0 T Siemens Harmony scanner. T2*weighted transversal Gradient Echo sequence (T2*GE) and a T2 weighted sagittal Turbospinecho sequence (T2TSE) visualised a hypointense linear margin of the upper cerebral vermis and the brain stem due to iron deposits. There was no cerebral vessel aneurysm visible.
A cerebral MRI was performed with a 1.0 T Siemens Harmony scanner. T2*weighted transversal Gradient Echo sequence (T2*GE) and a T2 weighted sagittal Turbospinecho sequence (T2TSE) visualised a hypointense linear margin of the upper cerebral vermis and the brain stem due to iron deposits. There was no cerebral vessel aneurysm visible.
Discussion
Superficial hemosiderosis of the central nervous system is a rare disease. Marginal deposition of haemosiderin in the cerebrum, cerebellum and spinal cord is caused by chronic subarachnoidal haemorrhage (SAH) [1, 2]. The diagnosis is made with brain and spinal MRI while CT is neither specific nor sensitive.
T2- and T2* weighted images delineate superficial hypointensities of spinal cord, medulla oblongata, pons, mesencephalon, cerebellum and cerebrum due to iron deposition [1, 2, 3].
Brain-tumours, vascular malformations and neurosurgery have been described as bleeding sources but these can be detecteded only in 50% of patients. The percentage of idiopathic haemosiderosis is therefore very high.
Clinical symptoms include deafness, cerebellar ataxia and pyramidal signs. CSF-investigation can give hints for chronic SAH, if levels of ferritin and iron are elevated.
Elimination of a bleeding source decelerates the clinical impairment but does not stop the disease. Therefore an early and extensive search for a bleeding source is highly favourable [2, 3, 4]. Besides treatment of the underlying condition a symptomatic treatment with antioxidants and radical scavengers has been discussed [3]. In our case a further investigation of the spinal cord was not possible because our examination was an order of another hospital.
T2- and T2* weighted images delineate superficial hypointensities of spinal cord, medulla oblongata, pons, mesencephalon, cerebellum and cerebrum due to iron deposition [1, 2, 3].
Brain-tumours, vascular malformations and neurosurgery have been described as bleeding sources but these can be detecteded only in 50% of patients. The percentage of idiopathic haemosiderosis is therefore very high.
Clinical symptoms include deafness, cerebellar ataxia and pyramidal signs. CSF-investigation can give hints for chronic SAH, if levels of ferritin and iron are elevated.
Elimination of a bleeding source decelerates the clinical impairment but does not stop the disease. Therefore an early and extensive search for a bleeding source is highly favourable [2, 3, 4]. Besides treatment of the underlying condition a symptomatic treatment with antioxidants and radical scavengers has been discussed [3]. In our case a further investigation of the spinal cord was not possible because our examination was an order of another hospital.
Differential Diagnosis List
Superficial cerebral hemosiderosis.
Final Diagnosis
Superficial cerebral hemosiderosis.
References
[1] Hillemanns A, Kastrup A, Kraph H (2001) H䀀mosiderose des Zentralnervensystems aufgrund rezidivierender Subarachnoidalblutungen bei myxopapill䀀ren Ependymom der Cauda equina. Rofo 173(9):851-3. (PMID: 11582568)
[2] Hug A, Hartmann M, Grau A (2004) Superfizielle Siderose des ZNS2 Kasuistiken und eine ܀bersicht über die Literatur. Nervenarzt 75: 584-8. (PMID: 15257381)
[3] Leussink VI, Flachenecker P, Brechtelsbauer D, Bendszus M, Sliwka U, Gold R, Becker G (2003) Superficial siderosis of the central nervous system: pathogenetic heterogeneity and therapeutic approaches. Acta Neurol Scand 107: 54-61. (PMID: 12542514)
[4] Maggioni F, Martinello F, Iavicoli R, Pietrogrande F, Trevisan CP (1994) Superficial hemosiderosis of the central nervous system. A case report. Acta Neurol (Napoli) 16: 134-41. (PMID: 7992663)
Case information
| URL: | https://www.eurorad.org/case/6941 |
| DOI: | 10.1594/EURORAD/CASE.6941 |
| ISSN: | 1563-4086 |
Figure 2
Tra T2*GE.
Hypointense margin is due to iron deposits.
Figure 3
T2*GE
Hypointense areas are visible at both thalami.
Figure 4
T2TSE sag
Sagittal images visualise black rim at the pons and brain stem.
Tra T2*GE
A hypointense small margin is visible at the pons and the upper cerebral vermis in comparison to a regular patient.
Tra T2*GE.
Hypointense margin is due to iron deposits.
T2*GE
Hypointense areas are visible at both thalami.
T2TSE sag
Sagittal images visualise black rim at the pons and brain stem.