Von Hippel-Lindau syndrome with microscopic hemangioblastomas of the spinal nerve roots

✓ The authors describe a case of von Hippel-Lindau syndrome diagnosed at autopsy in a 49-year-old woman. She died suddenly following hemorrhage from a cerebellar hemangioblastoma. Other autopsy findings included a retinal hemangioblastoma, an adrenal pheochromocytoma, and a clear-cell renal tumor. The case was distinguished by the unexpected finding of multiple microscopic hemangioblastomas of the spinal nerve roots. The case is discussed with emphasis on the incidence of spinal hemangioblastomas in von Hippel-Lindau syndrome.

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Lumbosacral intersegmental epispinal axons and ectopic ventral nerve rootlets

Journal of Neurosurgery ◽

10.3171/jns.1987.67.2.0269 ◽

1987 ◽

Vol 67 (2) ◽

pp. 269-277 ◽

Cited By ~ 9

Author(s):

Wesley W. Parke ◽

Ryo Watanabe

Keyword(s):

Nerve Root ◽

Sensory Nerve ◽

Conus Medullaris ◽

Spinal Nerve ◽

Nerve Fibers ◽

Ventral Horn ◽

Spinal Nerve Roots ◽

Nerve Roots ◽

Content Type ◽

Almost All

✓ An epispinal system of motor axons virtually covers the ventral and lateral funiculi of the human conus medullaris between the L-2 and S-2 levels. These nerve fibers apparently arise from motor cells of the ventral horn nuclei and join spinal nerve roots caudal to their level of origin. In all observed spinal cords, many of these axons converged at the cord surface and formed an irregular group of ectopic rootlets that could be visually traced to join conventional spinal nerve roots at one to several segments inferior to their original segmental level; occasional rootlets joined a dorsal nerve root. As almost all previous reports of nerve root interconnections involved only the dorsal roots and have been cited to explain a lack of an absolute segmental sensory nerve distribution, it is believed that these intersegmental motor fibers may similarly explain a more diffuse efferent distribution than has previously been suspected.

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Neurological and pathological effects of second lumbar spondylectomy and spinal column shortening in the dog

Journal of Neurosurgery ◽

10.3171/jns.1975.42.1.0047 ◽

1975 ◽

Vol 42 (1) ◽

pp. 47-58 ◽

Cited By ~ 5

Author(s):

Daniel J. Yturraspe ◽

William V. Lumb ◽

Stuart Young ◽

Harry A. Gorman

Keyword(s):

Vertebral Column ◽

Axonal Degeneration ◽

Spinal Column ◽

Lumbar Vertebra ◽

Spinal Nerve ◽

Spinal Nerve Roots ◽

Loss Of Function ◽

Nerve Roots ◽

Content Type ◽

Fine Print

✓ The second lumbar vertebra was surgically removed from 10 dogs, and the shortened vertebral column was stabilized by internal fixation with two types of plastic plates. Shortening of the spinal column was usually not associated with detectable loss of function or neurological deficit. Histological lesions, however, included widely disseminated axonal degeneration, gliosis, and atrophy of spinal nerve roots in the surgical area. The spinal cord adapted to shortening of the vertebral column by becoming intrinsically shorter, rather than by being displaced within the spinal canal.

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Multiple filum terminale hemangioblastomas symptomatic during pregnancy

Journal of Neurosurgery Spine ◽

10.3171/spi-07/08/254 ◽

2007 ◽

Vol 7 (2) ◽

pp. 254-258 ◽

Cited By ~ 15

Author(s):

Marta Ortega-Martínez ◽

Jose M. Cabezudo ◽

Ignacio Fernández-Portales ◽

Manuel Pineda-Palomo ◽

Jose Antonio Rodríguez-Sánchez ◽

...

Keyword(s):

Spinal Nerve ◽

Vascular Tumors ◽

Low Grade ◽

Filum Terminale ◽

Spinal Nerve Roots ◽

Nerve Roots ◽

Von Hippel Lindau

✓Hemangioblastomas are low-grade, highly vascular tumors commonly associated with von Hippel–Lindau (VHL) syndrome and most often appearing in the cerebellum. They very rarely occur in the spinal nerve roots, and an origin in the filum terminale is exceptional with no instances of multiple hemangioblastomas of the filum terminale reported in the literature. Because of their vascular nature, these lesions can enlarge and become symptomatic in the context of the changes that take place during pregnancy, as has been noted with cerebellar hemangioblastomas. In any case, the evolution of spinal hemangioblastomas during pregnancy is not well known given its rarity. The conjunction of both processes—that is, multiple hemangioblastomas arising in the filum terminale and pregnancy—is unique. The authors describe the case of a 41-year-old woman with multiple hemangioblastomas of the filum terminale and no other evidence of VHL syndrome, in whom pregnancy precipitated symptoms. The interruption of gestation led to a remission of the symptoms. The literature concerning filum terminale hemangioblastomas and pregnancy is also reviewed.

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Hemangioma of spinal nerve root

Journal of Neurosurgery Spine ◽

10.3171/spi.1999.91.2.0175 ◽

1999 ◽

Vol 91 (2) ◽

pp. 175-180 ◽

Cited By ~ 12

Author(s):

Federico Roncaroli ◽

Bernd W. Scheithauer ◽

William E. Krauss

Keyword(s):

Nerve Root ◽

Cauda Equina ◽

Spinal Nerve ◽

Nerve Fibers ◽

Capillary Hemangioma ◽

Spinal Nerve Root ◽

Spinal Nerve Roots ◽

Nerve Roots ◽

Content Type ◽

Fine Print

Object. Hemangiomas of the spinal nerve roots are rare. Only 12 cases have been reported in the literature, all since 1965. These lesions occurred in adults, primarily in males, and were located in the cauda equina. Of the 12 lesions, eight were cavernous hemangiomas. The authors report here the clinicopathological features of 10 cases of hemangioma of the spinal nerve root, nine of which were of the capillary type. Methods. The group included six men and four women who ranged in age from 40 to 62 years. The majority of patients presented with pain and weakness. One patient also experienced worsening of pain during menses. In all cases, gadolinium-enhanced magnetic resonance imaging or computerized tomography myelography demonstrated a discrete intraspinal extramedullary mass. Administration of contrast agent resulted in uniform, intense enhancement. A gross-total resection was achieved in all but one case; in most cases, it was necessary to sacrifice the parent nerve root. Preoperative symptoms and signs improved in nine cases. On histological examination, all but one tumor, a spindle-cell hemangioendothelioma, were shown to be ordinary capillary hemangiomas. Two lesions exhibited a cavernous component, and five showed a partly solid growth pattern resembling juvenile capillary hemangioma. In all cases, the relationship with the nerve root was histologically confirmed. The tumor—nerve relation varied. The intact nerve was displaced by the epineurial mass in three cases. The fascicles appeared separated in six cases, and individual nerve fibers were seen throughout one lesion with endoneurial involvement. Conclusions. Hemangiomas of spinal nerve roots pose a challenging diagnostic problem. Knowledge of their existence is relevant in that they may mimic tumors and are amenable to surgical treatment.

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