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Hemangioblastoma, case 2

CLINICAL HISTORY:
This 43-year old female patient is known to have von Hippel-Lindau disease. Fifteen years ago she underwent resection of a right cerebellar hemangioblastoma. For the last 2 years progressive walking problems occur. Now she complains of rapidly progressive gait instability, disequilibrium and weakness in all four extremities.

NEUROLOGICAL EXAMINATION: The gait is extremely spastic, broadbased and shuffling. There is a pronounced tendency to fall with severe dysmetria and dysdiadochokinesia. Paresis of all four extremities is noted, with hyperreflexia of the biceps and patellar tendon.

MRI:
a) CORONAL GADOLINIUM-ENHANCED SPIN-ECHO SEQUENCE OF THE CRANIOCERVICAL TRANSITION (IMAGES 1 - 2 - 3): A small enhancing nodule (arrowhead image 1) is seen in the left cerebellar hemisphere. In the cervical medulla, at the C2-C3 level, an intensely enhancing mass is seen. Above the lesion a cluster of serpiginous signal voids (arrow) is seen, representing draining veins. Enlargement of the spinal cord is noted.

b) SAGITTAL GD-ENHANCED SPIN-ECHO SEQUENCE (IMAGES 4 AND 5): The enhancing mass (arrowheads) and the draining veins are seen.

ANGIOGRAPHY:
a) ANTEROPOSTERIOR (IMAGE 6) AND LATERAL (IMAGE 7) PROJECTION OF THE LEFT VERTEBRAL ARTERY INJECTION, ARTERIAL PHASE: A hypervascular nodule is identified (arrowheads) in the left cerebellar hemisphere and the spinal cord.

b) ANTEROPOSTERIOR (IMAGE 8) AND LATERAL (IMAGE 9) PROJECTION OF THE RIGHT VERTEBRAL ARTERY INJECTION: An intense tumoral stain (arrowheads) is seen as are hypertrophic and tortuous draining veins (arrow).

RADIOLOGICAL DIAGNOSIS is cerebellar and spinal cord hemangioblastoma.

No surgical or pathological confirmation of these new lesions is available.

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Fig. 1

Hemangioblastoma, case 2, Fig. 1
Hemangioblastoma, case 2, Fig. 2
Hemangioblastoma, case 2, Fig. 3
Hemangioblastoma, case 2, Fig. 4
Hemangioblastoma, case 2, Fig. 5
Hemangioblastoma, case 2, Fig. 6
Hemangioblastoma, case 2, Fig. 7
Hemangioblastoma, case 2, Fig. 8
Hemangioblastoma, case 2, Fig. 9