Original Paper Eur Neurol 1992:32:270-273
Department of Neuroradiology. University of Turin, Italy
Keywords Central nervous system disease MR1 Supericial siderosis Chronic subarachnoid hemorrhage
Superficial Siderosis of the Central Nervous System: Neuroradiological Evaluation of Two Cases
Abstract We present 2 cases of superficial siderosis of the central nervous system sec ondary to chronic subarachnoid bleeding. The diagnosis was made with MR and cerebrospinal fluid (CSF) examination. MR showed, in T2-weighted images, superficial hypointensity of the cervical cord, brainstem, cerebellum, and basal cisterns due to hemosiderin deposits. CSF was xanthochromic, with increased protein content. In 1 of the 2 cases also the CT examination was positive, showing a hyperdense rim around the brainstem. A complete neuro radiological evaluation (MR, CT, angiography and myelography) did not per mit to detect the source of the bleeding.
Superficial siderosis of the central nervous system (CNS) is a rare condition consisting in intra- and extracel lular deposition of hemosiderin in the leptomeninges and in the outer layers of the brain, spinal cord and cranial nerves in contact with the cerebrospinal fluid (CSF). In most cases, it is due to the diffusion of iron pigment resulting from chronic subarachnoid bleeding. Clinical findings include progressive deafness, ataxia, dementia, dysfunction of some cranial nerves and of the pyramidal tracts [1, 2], Before the introduction of MR1. the diagnosis could only be suspected in life, based upon CSF and clinical findings, and could be confirmed only after autopsy [3]. With the support of MRI, a diagnostical tool for this condition in life is now available; hemosider in, due to its paramagnetic effect, can be easily depicted by this technique [4]. We report 2 cases of superficial siderosis, 1 of which is recognizable also by CT.
Received: April 26. 1991 Accepted August 28. 1991
Patients Case I G.F.. male, 56 years old. At the age of 35. relapsing palsies of the 7th cranial nerve: the last event. 4 years ago. was not totally recov ered. Three years ago. he started complaining of continuous head ache in the neck, often accompanied by nausea, vomiting, as well as by progressive bilateral hearing loss. He presented also slowly pro gressive dysphagia, especially for solid food, disturbance of balance, and ataxia worsened without visual feedback. At admission, neuro logical evaluation showed paraparcto-ataxic gait, with broad base and staggering gait, bilateral hearing loss, disphagia. upper limb atax ia. bright tendon reflexes and dysarthria. Upper functions were nor mal. CSF appeared bloody, xanthochromic after centrifugation; the red cell count was 700/mmT CSF proteins ranged up to 87 mg%. Oligoclonal bands were not found. CT was normal. MRI. performed in 1989 and 1990 showed, in T 2-weighted images, a superficial hypointensity of the vermis, brainstem and of the superficial layers of the cervical cord (fig. 1). Vermis and spinal cord were slightly atrophic.
G.B. Bradac. MD Department o f Neuroradiology University of Turin Via Cherasco 15,1-10126 Torino (Italy)
© 1992 S. Karger AG. Basel 0014-3022/92/0325-0270 $2.75/0
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 7/12/2017 6:08:16 AM
D. Dciniele M. Bracchi A. Riva S. Ditca G. Stura G. B. Bradac
A carotid and vertebral angiography as well as a myelography did not show vascular malformations or other lesions responsible for hemosiderin staining.
Case 2 F.L.. male. 49 years old. Since the age of 40. the patient had devel oped a progressive hearing loss till total deafness. In the last 3 years he also had progressive balance disturbance with ataxic gait. At the time of this study he was unable to walk without support. Since a few years, kinetic tremor had been present throughout intended arm movement. Neurological examination showed ataxia o f stance, ataxic and staggering gait, dysdiadochokinesia. Except for the hear ing loss, the cranial nerves were normal. Upper functions and mem ory were normal. CSF appeared clear, xanthochromic, red cell count w'as 430/mm-,. Proteins were 68 mg%; oligoclonal bands were not found. CT showed a thin, hyperdense rim around the brainstem, espe cially visible around the midbrain: a diffuse atrophy w'as also visible (fig. 2). MRI showed marked atrophy of the cerebellum, brainstem and cervical cord. In Ti-weighted images, a hypointense rim was evident in the superficial layers of the brainstem and cerebellum, mainly at the level of the upper vermis (fig. 3). in the medial temporal cisterns, in the silvian fissures, in the interhemispheric fissure and in the retropulvinar and calcarine fissures. A similar finding w'as seen around the whole spinal cord and at the bottom of the dural sac (fig. 4). Carotid and vertebral angiography and myelography did not show lesions responsible for the hemosiderosis in this case, either.
Fig. 1. MR. sagittal. SE. Ti-weighted image. TR = 2.000. TE = 100. Hypotcnsity of the surfaces of the medulla, the spinal cord, the quadrigeminal plate and the vermian cisterns.
Discussion
Fig. 2. Plain CT. Hyperintensc rim around the medulla and the mesencephalon.
271
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 7/12/2017 6:08:16 AM
Superficial siderosis is a rare condition with only a few cases described in the literature [ 1-5]. Clinical and experimental findings indicate that the deposition of hemosiderin is due to a chronical bleeding into CSF; a unique subarachnoid bleeding does not play any role in determining this illness [1-3]. Systemic hemo chromatosis has no relationship with superficial hemo siderosis of CNS [2, 3]. In our 2 cases too. neither can point out any sure subarachnoid hemorrhage, nor any clinical finding suggesting a systemic hemochromatosis. Experimental findings by Ivanowski and Olzewski [3] reproduced a similar neuropathological feature only after repeated subarachnoid injections of blood-, hemoglobinand iron-containing substances. This experiment con firms that repeated bleedings are necessary. It is often impossible to demonstrate any lesion which might be thought responsible of chronical bleeding; in the rare cases in which it has been identified it resulted of vascular (aneurysm, arterovenous malformations) or neo plastic (ependymoma, oligodendroglioma, meningioma, meningeal carcinomatosis) nature [2].
Fig. 3. MR. axial. SE,T;-weighted image, TR = 2,000. TE = 70. Hvpointense rim sur rounding the medulla, the mesencephalon and the supravermian cistern.
272
Fig. 4. MR, sagittal. SE. T^-weighted image. TR = 2.000. TE = 100. Hypointensity around the spinal cord and at the bottom of the dural sac.
even in our study, performed with a 0.5-T device, the sig nal changes were markedly evident. CT is usually reported to be negative [5]; nevertheless in 1 of our 2 cases, hemosiderin deposits were so heavy that CT could demonstrate them as a hvperdense rim around the brainstem.
Daniele/Bracchi/Riva/Duca/Stura/Bradac
Superficial Siderosis of CNS
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 7/12/2017 6:08:16 AM
Hemoglobin deposits are found mostly in the pial layers of the spinal cord, pons and medulla oblongata, the anterior third of the cerebellum, basal cisterns. Distribu tion of blood deposits may be related to CSF dynamics. The proximal segments of cranial nerves, i.e., those in contact with CSF, are often pigmented. The eighth pair is particularly affected, perhaps because of the length of the cisternal tract [1], Impairment of optical nerves with visual loss some times occurs [2], Dementia is described to be one of the main late symptoms; however, in our cases it has not been observed perhaps because of the early diagnosis allowed by MRI. Until the introduction of MRI. the diagnosis of hemo siderosis had been considered exclusively autoptic [1], In the literature, we have found only 1 case in which it had been suspected in life [ 1]. Even recent observations affirm that CT is completely inadequate to confirm hemosidero sis [5]. With MRI. the diagnosis is relatively easy. The hypointensity in Ti-weighted sequences is caused by iron-containing blood pigments which marked the paramagnetic effect. These pigments are stored in astro cytes and macrophages; the intracellular distribution in creases the paramagnetic effect. The favorite sites for iron storage are basal structures: in MRI deposits on the quadrigeminal plate and on the upper vermis are particularly remarkable. Previous studies were performed with 1.5-T devices, more sensitive to the paramagnetic effect [4. 5]; however.
In both cases we studied, it was not possible to identify the bleeding source, even with complete angiographic and myélographie studies. A hypothesis could be the presence of cryptic vascular malformations, possibly on the capillary or venous dis trict.
In conclusion, superficial siderosis of CNS is a rare condition, slowly progressive, but anyway characterized by severe prognosis. The clinician examining a patient with progressive hearing loss and cerebellar signs should be aware that these symptoms can be secondary to super ficial siderosis of CNS, and that MRI can easily distin guish this form from other degenerative diseases.
References
2
Shcrwin I, Toll K: Superficial siderosis of the central nervous system. Dis Cent Nerv Syst 1972:6:413-417. ’ Tomlinson BE. Walton JN: Superficial hacmasidcrosis o f the central nervous system. J Neu rol Neurosurg Psychiatry 1964:27:332-339.
3
4
Ivanovski K. Olzewski J: The effect of sub arachnoid injections of iron containing sub stances on the central nervous system. J Neuropathol Exp Neurol 1960:19:433-448. Gomori J. Grossmann RJ. Bilaniuk LT. Zim merman RA. Goldberg HI: High field MRI of superficial siderosis of central nervous system. J Comput Assist Tomogr 1985:9/5:972-975.
5
Zimmermann R. Hcssclink JR. Bilaniuk LT, et al: Bilateral pial siderosis and hearing loss: Syn drome with negative CT and positive high field MR findings. Radiology 1988:169/P:312.
273
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 7/12/2017 6:08:16 AM
1
Department of Neuroradiology. University of Turin, Italy
Keywords Central nervous system disease MR1 Supericial siderosis Chronic subarachnoid hemorrhage
Superficial Siderosis of the Central Nervous System: Neuroradiological Evaluation of Two Cases
Abstract We present 2 cases of superficial siderosis of the central nervous system sec ondary to chronic subarachnoid bleeding. The diagnosis was made with MR and cerebrospinal fluid (CSF) examination. MR showed, in T2-weighted images, superficial hypointensity of the cervical cord, brainstem, cerebellum, and basal cisterns due to hemosiderin deposits. CSF was xanthochromic, with increased protein content. In 1 of the 2 cases also the CT examination was positive, showing a hyperdense rim around the brainstem. A complete neuro radiological evaluation (MR, CT, angiography and myelography) did not per mit to detect the source of the bleeding.
Superficial siderosis of the central nervous system (CNS) is a rare condition consisting in intra- and extracel lular deposition of hemosiderin in the leptomeninges and in the outer layers of the brain, spinal cord and cranial nerves in contact with the cerebrospinal fluid (CSF). In most cases, it is due to the diffusion of iron pigment resulting from chronic subarachnoid bleeding. Clinical findings include progressive deafness, ataxia, dementia, dysfunction of some cranial nerves and of the pyramidal tracts [1, 2], Before the introduction of MR1. the diagnosis could only be suspected in life, based upon CSF and clinical findings, and could be confirmed only after autopsy [3]. With the support of MRI, a diagnostical tool for this condition in life is now available; hemosider in, due to its paramagnetic effect, can be easily depicted by this technique [4]. We report 2 cases of superficial siderosis, 1 of which is recognizable also by CT.
Received: April 26. 1991 Accepted August 28. 1991
Patients Case I G.F.. male, 56 years old. At the age of 35. relapsing palsies of the 7th cranial nerve: the last event. 4 years ago. was not totally recov ered. Three years ago. he started complaining of continuous head ache in the neck, often accompanied by nausea, vomiting, as well as by progressive bilateral hearing loss. He presented also slowly pro gressive dysphagia, especially for solid food, disturbance of balance, and ataxia worsened without visual feedback. At admission, neuro logical evaluation showed paraparcto-ataxic gait, with broad base and staggering gait, bilateral hearing loss, disphagia. upper limb atax ia. bright tendon reflexes and dysarthria. Upper functions were nor mal. CSF appeared bloody, xanthochromic after centrifugation; the red cell count was 700/mmT CSF proteins ranged up to 87 mg%. Oligoclonal bands were not found. CT was normal. MRI. performed in 1989 and 1990 showed, in T 2-weighted images, a superficial hypointensity of the vermis, brainstem and of the superficial layers of the cervical cord (fig. 1). Vermis and spinal cord were slightly atrophic.
G.B. Bradac. MD Department o f Neuroradiology University of Turin Via Cherasco 15,1-10126 Torino (Italy)
© 1992 S. Karger AG. Basel 0014-3022/92/0325-0270 $2.75/0
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 7/12/2017 6:08:16 AM
D. Dciniele M. Bracchi A. Riva S. Ditca G. Stura G. B. Bradac
A carotid and vertebral angiography as well as a myelography did not show vascular malformations or other lesions responsible for hemosiderin staining.
Case 2 F.L.. male. 49 years old. Since the age of 40. the patient had devel oped a progressive hearing loss till total deafness. In the last 3 years he also had progressive balance disturbance with ataxic gait. At the time of this study he was unable to walk without support. Since a few years, kinetic tremor had been present throughout intended arm movement. Neurological examination showed ataxia o f stance, ataxic and staggering gait, dysdiadochokinesia. Except for the hear ing loss, the cranial nerves were normal. Upper functions and mem ory were normal. CSF appeared clear, xanthochromic, red cell count w'as 430/mm-,. Proteins were 68 mg%; oligoclonal bands were not found. CT showed a thin, hyperdense rim around the brainstem, espe cially visible around the midbrain: a diffuse atrophy w'as also visible (fig. 2). MRI showed marked atrophy of the cerebellum, brainstem and cervical cord. In Ti-weighted images, a hypointense rim was evident in the superficial layers of the brainstem and cerebellum, mainly at the level of the upper vermis (fig. 3). in the medial temporal cisterns, in the silvian fissures, in the interhemispheric fissure and in the retropulvinar and calcarine fissures. A similar finding w'as seen around the whole spinal cord and at the bottom of the dural sac (fig. 4). Carotid and vertebral angiography and myelography did not show lesions responsible for the hemosiderosis in this case, either.
Fig. 1. MR. sagittal. SE. Ti-weighted image. TR = 2.000. TE = 100. Hypotcnsity of the surfaces of the medulla, the spinal cord, the quadrigeminal plate and the vermian cisterns.
Discussion
Fig. 2. Plain CT. Hyperintensc rim around the medulla and the mesencephalon.
271
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 7/12/2017 6:08:16 AM
Superficial siderosis is a rare condition with only a few cases described in the literature [ 1-5]. Clinical and experimental findings indicate that the deposition of hemosiderin is due to a chronical bleeding into CSF; a unique subarachnoid bleeding does not play any role in determining this illness [1-3]. Systemic hemo chromatosis has no relationship with superficial hemo siderosis of CNS [2, 3]. In our 2 cases too. neither can point out any sure subarachnoid hemorrhage, nor any clinical finding suggesting a systemic hemochromatosis. Experimental findings by Ivanowski and Olzewski [3] reproduced a similar neuropathological feature only after repeated subarachnoid injections of blood-, hemoglobinand iron-containing substances. This experiment con firms that repeated bleedings are necessary. It is often impossible to demonstrate any lesion which might be thought responsible of chronical bleeding; in the rare cases in which it has been identified it resulted of vascular (aneurysm, arterovenous malformations) or neo plastic (ependymoma, oligodendroglioma, meningioma, meningeal carcinomatosis) nature [2].
Fig. 3. MR. axial. SE,T;-weighted image, TR = 2,000. TE = 70. Hvpointense rim sur rounding the medulla, the mesencephalon and the supravermian cistern.
272
Fig. 4. MR, sagittal. SE. T^-weighted image. TR = 2.000. TE = 100. Hypointensity around the spinal cord and at the bottom of the dural sac.
even in our study, performed with a 0.5-T device, the sig nal changes were markedly evident. CT is usually reported to be negative [5]; nevertheless in 1 of our 2 cases, hemosiderin deposits were so heavy that CT could demonstrate them as a hvperdense rim around the brainstem.
Daniele/Bracchi/Riva/Duca/Stura/Bradac
Superficial Siderosis of CNS
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 7/12/2017 6:08:16 AM
Hemoglobin deposits are found mostly in the pial layers of the spinal cord, pons and medulla oblongata, the anterior third of the cerebellum, basal cisterns. Distribu tion of blood deposits may be related to CSF dynamics. The proximal segments of cranial nerves, i.e., those in contact with CSF, are often pigmented. The eighth pair is particularly affected, perhaps because of the length of the cisternal tract [1], Impairment of optical nerves with visual loss some times occurs [2], Dementia is described to be one of the main late symptoms; however, in our cases it has not been observed perhaps because of the early diagnosis allowed by MRI. Until the introduction of MRI. the diagnosis of hemo siderosis had been considered exclusively autoptic [1], In the literature, we have found only 1 case in which it had been suspected in life [ 1]. Even recent observations affirm that CT is completely inadequate to confirm hemosidero sis [5]. With MRI. the diagnosis is relatively easy. The hypointensity in Ti-weighted sequences is caused by iron-containing blood pigments which marked the paramagnetic effect. These pigments are stored in astro cytes and macrophages; the intracellular distribution in creases the paramagnetic effect. The favorite sites for iron storage are basal structures: in MRI deposits on the quadrigeminal plate and on the upper vermis are particularly remarkable. Previous studies were performed with 1.5-T devices, more sensitive to the paramagnetic effect [4. 5]; however.
In both cases we studied, it was not possible to identify the bleeding source, even with complete angiographic and myélographie studies. A hypothesis could be the presence of cryptic vascular malformations, possibly on the capillary or venous dis trict.
In conclusion, superficial siderosis of CNS is a rare condition, slowly progressive, but anyway characterized by severe prognosis. The clinician examining a patient with progressive hearing loss and cerebellar signs should be aware that these symptoms can be secondary to super ficial siderosis of CNS, and that MRI can easily distin guish this form from other degenerative diseases.
References
2
Shcrwin I, Toll K: Superficial siderosis of the central nervous system. Dis Cent Nerv Syst 1972:6:413-417. ’ Tomlinson BE. Walton JN: Superficial hacmasidcrosis o f the central nervous system. J Neu rol Neurosurg Psychiatry 1964:27:332-339.
3
4
Ivanovski K. Olzewski J: The effect of sub arachnoid injections of iron containing sub stances on the central nervous system. J Neuropathol Exp Neurol 1960:19:433-448. Gomori J. Grossmann RJ. Bilaniuk LT. Zim merman RA. Goldberg HI: High field MRI of superficial siderosis of central nervous system. J Comput Assist Tomogr 1985:9/5:972-975.
5
Zimmermann R. Hcssclink JR. Bilaniuk LT, et al: Bilateral pial siderosis and hearing loss: Syn drome with negative CT and positive high field MR findings. Radiology 1988:169/P:312.
273
Downloaded by: Karolinska Institutet, University Library 130.237.122.245 - 7/12/2017 6:08:16 AM
1
