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Sudden Paraplegia in a Patient With Chronic Myelopathy

Two Faces of Spinal Cavernous Hemangioma

Sung, Joo Hye MD; Baek, Seol-Hee MD; Kim, Byung-Jo MD, PhD

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American Journal of Physical Medicine & Rehabilitation: April 2020 - Volume 99 - Issue 4 - p e56-e57
doi: 10.1097/PHM.0000000000001320
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A 49-yr-old man presented with a 1-yr history of relapsing and remitting sensory deficits of the legs. His sensory loss and paresthesia started at his right toe and subsequently spread to the opposite side 6 mos later. The symptoms initially resolved 3 mos before presentation. However, 3 wks before the hospital visit, the sensory symptoms relapsed, progressing to below the T10 dermatome level. Physical examination revealed decreased sensation in all sensory modalities below the umbilicus. Bilateral lower limbs showed slight proximal weakness (motor grade 4). Deep tendon reflexes were hyperactive and pathologic reflexes were positive in both legs. Thoracic spinal magnetic resonance imaging (MRI) revealed an oval-shaped intramedullary mass at the T8–T9 level as well as longitudinally extensive myelitis along T6–T10 (Fig. 1A–D). After 5 days of steroid pulse therapy, sensory symptoms improved to a comfortable level. However, a week later, motor function of his lower extremity deteriorated suddenly to grade 1-2/5. Follow-up MRI showed a T2-weighted dark signal around the intramedullary mass, suggestive of new bleeding (Fig. 1E). Immediate surgical intervention led to partial recovery. Histological examination confirmed cavernous hemangioma (Fig. 2).

FIGURE 1
FIGURE 1:
A–C, Axial T2-weighted images show cord signal change adjacent to the hemangioma (A), transverse section of cavernous hemangioma (B), and hemosiderin cap (C). D, Sagittal T2-weighted image shows classic “mulberry-like” heterogenous hyperintensity with myelitis along T6–T10. E, Note the dark signal (arrows) on the follow-up MRI after symptom aggravation.
FIGURE 2
FIGURE 2:
A, Photomicrographs (hematoxylin and eosin stains) of cavernous hemangioma (bar = 200 μm) reveal closely apposed, dilated, thin-fibrous-walled vascular channels with a single endothelial cell-lining and shared common wall, without intervening neuroglial tissue. B, Neighboring spinal cord shows gliosis, old hemorrhages with aggregates of hemosiderin-laden macrophages (arrows, bar = 100 μm) and extensive fresh hemorrhages (arrows, bar = 200 μm) (C).

Spinal cavernous hemangioma is a rare vascular malformation, which accounts for approximately 5% of all cavernous malformation and 5%–12% of spinal cord vascular lesions.1 It is a grossly well-circumscribed, mulberry-like lesion, microscopically characterized by dilated sinusoidal channels of dense capillaries.

Spinal cord cavernous hemangioma can have two discrete presentation modes: rapid neurological decline and progressive myelopathy.2 The former occurs secondary to frank hemorrhage within the spinal cord, whereas the latter results from microhemorrhages and reactive gliotic changes.3 On MRI, the deposition of hemosiderin resulting in a hypointense rim around the mixed signal intensity is the pathognomonic appearance of cavernous hemangioma. However, cavernous hemangiomas may have a more homogeneous hyperintense or hypointense appearance, which can be confused with demyelinating diseases, such as neuromyelitis optica spectrum disorder, especially with clinical histories of recurrent or progressive neurological decline.1 This case showed both patterns of clinical presentation during the clinical course, demonstrated by the sequential changes in imaging features on MRI. Despite the initial clinical picture and concurrent existence of longitudinally extensive myelitis, massive hemorrhage was identified around the intramedullary mass, which caused the abrupt symptom aggravation.

Spinal cavernous hemangioma should not be missed in the differential diagnosis of intramedullary lesions. Identifying spinal cavernous hemangioma is important, as early surgical removal is essential in cases of progressive or severe neurologic compromise.

REFERENCES

1. Gross BA, Du R, Popp AJ, et al: Intramedullary spinal cord cavernous malformations. Neurosurg Focus 2010;29:E14
2. Deutsch H, Jallo GI, Faktorovich A, et al: Spinal intramedullary cavernoma: clinical presentation and surgical outcome. J Neurosurg Spine 2000;93:65–70
3. Ogilvy CS, Louis DN, Ojemann RG: Intramedullary cavernous angiomas of the spinal cord: clinical presentation, pathological features, and surgical management. Neurosurgery 1992;31:219–30
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