Correspondence

Cavernous Angioma of the Brain Stem Mimicking Multiple Sclerosis

N Engl J Med 1997; 336:875-876March 20, 1997

Article

To the Editor:

Vascular malformations of the brain stem are relatively uncommon and represent about 7 percent of all vascular malformations of the brain.1 They may be manifested as subarachnoid or intraparenchymatous hemorrhages, seizures, or fluctuating or slowly progressive neurologic deficit. In 50 percent of cases, vascular malformations of the brain stem are not visible with angiography.1 Also, standard computed tomography (CT) of the brain may fail to detect small vascular malformations of the brain stem, whereas magnetic resonance imaging (MRI) offers better sensitivity. There are case reports of vascular malformations simulating multiple sclerosis that are revealed after many years by MRI, CT, or angiography.2,3 In these patients, however, analyses of cerebrospinal fluid were normal. We describe a patient with fluctuating, progressive neurologic deficit and a cavernous angioma of the brain stem that was revealed only by MRI. For 15 years, this patient was believed to have multiple sclerosis because of fluctuating neurologic symptoms, negative CT of the brain, and the presence of oligoclonal bands in the cerebrospinal fluid.

This 35-year-old man was admitted in 1979 because of the sudden onset of rotatory vertigo, nausea, vomiting, and paresthesias on the left side of the face. Clinical examination revealed slow beating nystagmus to the left, slight incoordination of the left arm, and left-sided paresis in the distribution of the glossopharyngeal nerve. Left-sided hypesthesia of the face and neck and decreased corneal reflex were also noted. CT of the brain was normal. Cerebrospinal-fluid analysis revealed three oligoclonal bands and an elevated IgG index but normal red-cell and white-cell counts. Visual evoked potentials were normal. The symptoms disappeared within two months, except for a slight numbness on the left side of the face. In 1983, the patient reported having headache and increased numbness on the left side of the face and the left arm. He also had intermittent hiccups. Neurologic examination showed a distinct ataxia and incoordination of the left arm. Cerebrospinal-fluid analysis revealed eight oligoclonal bands, a normal IgG index and white-cell count, and a slightly increased albumin ratio. After two months, the symptoms were restricted to a slight numbness of the left side of the face and the left arm. A diagnosis of probable multiple sclerosis was made. In 1993, numbness of the left side of the body progressed and intermittent hiccups reappeared. The hiccups disappeared after six months. A year later the same symptoms returned. Lumbar puncture showed seven to nine oligoclonal bands and an increased IgG index in the cerebrospinal fluid. MRI showed a vascular malformation typical of cavernous angioma in the brain stem that was 15 mm in diameter (Figure 1AFigure 1Cavernous Angioma in a 35-Year-Old Man with Symptoms Mimicking Multiple Sclerosis. and Figure 1B). Surgical removal of the angioma was carried out without complications. Pathological examination showed dilated, closed, packed blood vessels typical of cavernous angioma. A neurologic examination two months later showed persistent slight left-sided ataxia and decreased sensitivity to touch and pain; otherwise, the patient was well. He was able to return to work. On the last visit, in August 1996, the left-sided ataxia had decreased but a slight left-sided hypesthesia persisted.

A fluctuating clinical course simulating multiple sclerosis is rare but has been described in case reports of patients with vascular malformations of the brain stem.1-3 In a recent report,4 multiple sclerosis was initially diagnosed in 8 of 21 patients with occult cerebrovascular malformations of the brain stem. All had undergone CT of the brain, and 14 of the 21 patients had undergone vertebral angiography with negative or questionable results. MRI allowed a diagnosis to be made with sufficient certainty in all cases. To our knowledge, no patient in any study had oligoclonal bands in the cerebrospinal fluid, as seen in our patient. Even though oligoclonal bands and an elevated IgG index in the cerebrospinal fluid are a sensitive marker for multiple sclerosis, they are not specific to the disease and may occur in patients with other neurologic diseases.

The fluctuations of symptoms may be caused by small hemorrhages or, in some cases, by mechanical compression or ischemia due to a slowly enlarging vascular malformation. The diagnosis of multiple sclerosis may be made in as many as 10 percent of patients. The use of MRI has probably reduced this patient group, although the detection of multiple small white-matter lesions is not specific to multiple sclerosis. No such areas were discovered in our patient.

Despite a relatively low risk of bleeding (annualized bleeding rate, 0.7 percent5), surgical treatment has been proposed for all but asymptomatic patients with high surgical risk. In our patient, successful removal of a cavernous angioma intrinsic to the medulla oblongata was performed.

Magnus Vrethem, M.D., Ph.D.
Karl-Åke Thuomas, M.D., Ph.D.
Jan Hillman, M.D., Ph.D.
University Hospital, S-581 85 Linköping, Sweden

5 References

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Citing Articles (2)

Citing Articles

1. 1

   Bradley A. Gross, H. Hunt Batjer, Issam A. Awad, Bernard R. Bendok. (2009) BRAINSTEM CAVERNOUS MALFORMATIONS. Neurosurgery 64:5, E805-E818
   CrossRef

2. 2

   Magnus Vrethem, Leif Davidsson, Julia Bak. (1998) Intracerebral malignant lymphoma in a patient with long-standing multiple sclerosis. European Journal of Neurology 5:5, 507-510
   CrossRef