Clinical review

Cavernous haemangioma mimicking multiple sclerosis

Department of Neurology, Queen Elizabeth Hospital, Birmingham B15 2TT

Correspondence to: Dr Winer j.b.winer{at}bham.ac.uk

Patients diagnosed with multiple sclerosis before the advent of magnetic resonance imaging and whose symptoms could be attributable cavernous haemangioma should be reviewed with magnetic resonance imaging

Cavernous haemangiomas are vascular malformations that rarely affect the brain, but their clinical presentation can simulate multiple sclerosis. The likelihood of mistaking cavernous haemangioma for multiple sclerosis is increased further by the fact that cavernous haemangiomas are poorly identified by angiography, and even computed tomography has a relatively low sensitivity and specificity for these lesions.¹ However, high field magnetic resonance imaging is able to distinguish cavernous haemangiomas, and since its introduction increasing numbers have been reported.¹

We describe two patients who had been diagnosed as having multiple sclerosis many years before the widespread use of computed tomography or the advent of magnetic resonance imaging. Magnetic resonance imaging subsequently showed that they had cavernous haemangioma.

	Case reports

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Case 1
A 17 year old boy presented in 1975 with diplopia, left sided facial weakness, and dizziness. He was right handed. Physical examination showed that he had left VI nerve palsy and mild weakness of the lower left side of his face. The patient was admitted to hospital for further investigation. A lumbar puncture showed clear cerebrospinal fluid under normal pressure but a slightly high  globulin fraction (0.04 g/l; 12.1% of total protein), a red cell count of 4×10⁹/l, and a white cell count of 1×10⁹/l.

Case 2
A 36 year old man presented to a neurologist in 1964. He was right handed. He had a five year history of laughing without reason (which had led to considerable embarrassment) and increasing weakness of the right arm and leg. Physical examination showed that he had a right spastic hemiparesis and a pseudobulbar palsy. No abnormalities were detected by a radioisotope scan or angiography of the brain, and multiple sclerosis was considered the most likely diagnosis.

View larger version (109K): [in this window] [in a new window]   Magnetic resonance imaging scan (T2 weighted sagittal section) of a large cavernous haemangioma in the upper brain stem of case 1

	Discussion

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Cavernous haemangiomas are an irregular honeycomb of thin-walled vascular spaces without intervening brain tissue, and usually without any increase in the vascularity of adjacent tissues. The lesions generally occur singly. Only a few are infratentorial, and the pons is the commonest site in these cases. Cavernous haemangiomas usually manifest with the effects of a space occupying lesion or from spontaneous haemorrhage.²

However, there have been several published reports of vascular malformations of the brain stem that run a long, insidious course and simulate multiple sclerosis.^3-5 Cavernous haemangiomas are not usually visualised by angiography because the blood flow through them is slow.⁶ Nor is computed tomography able to identify reliably all cavernous haemangiomas. A misdiagnosis of multiple sclerosis was therefore a particular hazard before magnetic resonance imaging became widely available.

It is interesting that patient 1 had a high  globulin fractiona finding that would be consistent with multiple sclerosis. A recent case report describes a patient who was thought to have multiple sclerosis on the basis of an insidious history, negative computed tomographic findings, cerebrospinal fluid with oligoclonal bands, and (later) a raised  globulin fraction.³ These authors noted that oligoclonal bands and raised  globulin values are sensitive, but not specific, for multiple sclerosis.

The clinical distinction between cavernous haemangiomas and multiple sclerosis affecting the brain stem is sometimes difficult. In neither of our patients was there an obvious episode of optic neuritis or any disordered eye movement other than the VI nerve palsyfindings that would have been unusual for multiple sclerosis. In both our patients, the initial clinical picture could have been attributed to a single brain stem lesion. In these cases there should be a high index of suspicion for a structural lesion. However, cavernous haemangioma can occasionally be multiple. This adds to the difficulty in making a diagnosis and supports the value of magnetic resonance imaging in confirming a diagnosis of multiple sclerosis.

Abnormal findings in cerebrospinal fluid and results of electrophysiological studies are clearly non-specific. However, with the increasing use of magnetic resonance imaging for detecting white matter plaques when multiple sclerosis is suspected, differentiating between the two abnormalities should not pose a problem. For patients diagnosed as having multiple sclerosis before the advent of modern imaging technologies, particularly those whose symptoms could be attributable to single lesions, the diagnosis should be reviewed carefully.

	Acknowledgments

We thank Dr R Hughes and Professor A C Williams.

	References

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1.	Curling OD, Kelly DL, Elster AD, Craven TE. An analysis of the natural history of cavernous angioma. J Neurosurg 1991; 75: 702-708[Medline].
2.	Russell DS, Rubinstein LJ. Pathology of tumours of the nervous system. 5th ed. London: Edward Arnold , 1989.
3.	Vrethem M, Thomas KA, Hillman J. Cavernous angioma of the brain stem mimicking multiple sclerosis [letter]. N Engl J Med 1997; 336: 875-876[Free Full Text].
4.	Honczarenko K, Fryze C, Nowacki P, Osuch Z, Grzelec H, Fabian A. Cavernous angioma of brain stem mimicking multiple sclerosis. Folia Neuropatholigica 1995; 33: 251-254.
5.	Stahl SM, Johnson KP, Malamud N. The clinical and pathological spectrum of brain stem vascular malformations. Long term course stimulates multiple sclerosis. Arch Neurol 1980; 37: 25-29[Abstract].
6.	Requena I, Arias M, Lopez-Ibor L, Pereiro I, Barba A, Alonso A, et al. Cavernomas of the central nervous system: clinical and neuroimaging manifestations in 47 patients. J Neurol Neurosurg Psychiatry 1991; 54: 590-595[Abstract].

(Accepted 9 November 1998)