Multiple Hereditary Hemangioblastomas of the Nervous System

Levin, Paul M.

doi:10.1001/archneurpsyc.1936.02260080156012

Cited by 28 publications

(5 citation statements)

References 14 publications

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“…The prevalence of spinal hemangioblastomas has been reported in several studies. 7,12,15,24,28) Prior to the introduction of MR imaging, 24) spinal lesions were more commonly found as sporadic isolated le-Spinal Hemangioblastomas in Sporadic Disease and VHL Syndrome sions (15%) than in association with VHL syndrome (7%), whereas a more recent study 7) has shown exactly opposite findings (12% and 47%, respectively). An earlier autopsy series revealed a high incidence of spinal lesions in patients with VHL syndrome.…”

Section: Discussionmentioning

confidence: 99%

“…An earlier autopsy series revealed a high incidence of spinal lesions in patients with VHL syndrome. One study found that spinal lesions were present in every 10 proven cases of VHL syndrome, 15) and another showed that multiple small miliary lesions were present in the spinal cord and nerve roots in patients with VHL syndrome. 12,28) Our study found that spinal lesions were much more prevalent in patients with VHL syndrome (88.2%) than in those with sporadic disease (20.5%, p º 0.001).…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Comparative Analysis of Spinal Hemangioblastomas in Sporadic Disease and Von Hippel-Lindau Syndrome

Takai

Taniguchi

Takahashi

et al. 2010

Neurol. Med. Chir.(Tokyo)

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The clinical differences were evaluated in spinal hemangioblastoma between patients with sporadic disease and patients with von Hippel-Lindau (VHL) syndrome. The distribution of hemangioblastomas in the central nervous system was investigated in 56 patients treated between 1988 and 2008 at the University of Tokyo Hospital. The characteristics of spinal hemangioblastomas were compared in 35 patients including 17 with sporadic disease and 18 with VHL syndrome treated between 1988 and 2008 at our hospital and three affiliated institutions. Spinal hemangioblastomas were much more prevalent in patients with VHL syndrome (88.2%) than in patients with sporadic disease (20.5%, p º 0.001). Spinal hemangioblastomas associated with VHL syndrome were diagnosed a decade earlier (p ＝ 0.007) and were associated with less severe neurological symptoms than sporadic lesions (p ＝ 0.004). Most patients with sporadic disease had a single lesion at the cervical or thoracic level, whereas patients with VHL syndrome had multiple lesions at all spinal levels (p ＝ 0.04). Patients with sporadic disease exhibited significant improvement in postoperative neurological status (p ＝ 0.02), whereas patients with VHL syndrome had no change in status (p ＝ 1.00). Number of removed lesions (p ＝ 0.03) and lower spinal cord lesions (p ＝ 0.05) were associated with poor surgical outcome. Tumor recurrence was correlated with partial removal of lesions (p ＝ 0.05). One third of patients with VHL syndrome developed new lesions every 2 years. The major finding of this study is that the incidence of spinal hemangioblastoma, distributed through all spinal levels, may be as high as 88% in patients with VHL syndrome, which is much greater than previously reported.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Comparative Analysis of Spinal Hemangioblastomas in Sporadic Disease and Von Hippel-Lindau Syndrome

Takai

Taniguchi

Takahashi

et al. 2010

Neurol. Med. Chir.(Tokyo)

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“…6 There are reports of less than 100 spinal hemangioblastomas in von Hippel-Lindau's complex, and these are said to have a predilection for cervico-dorsal and dorso-lumbar regions. In patients with von Hippel-Lindau's disease, symptomatic hemangioblastomas have occurred in the cerebrum, ~ brain stem, 8 spinal cord, a,2,4,5,7,9,1~ and spinal nerve roots.…”

Section: Discussionmentioning

confidence: 99%

Familial von Hippel-Lindau's Disease

Wright¹

1969

Journal of Neurosurgery

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“…It was Arvid Lindau who in 1926 proved the hemangiomatous nature of certain cystic tumors of the cerebellum and showed that these tumors are frequently associated with so-called angiomatosis retinae (von Hippel's disease).1 The familial incidence of this condition was understood by Lindau but was definitely established by M\l=o"\ller, who reported six cases (four of them verified) in three generations of a single family.2 Levin noted that, in every reported case of multiple vascular tumors of the nervous system with autopsy examination, a similar lesion was found in the spinal cord. 3 Wyburn-Mason reviewed 47 cases and noted that few cases affecting the spinal cord have shown familial or hereditary influences but found a definite association with hypernephroma, apparently of a nonmalignant type.4 Various lesions occur with lesser frequency, including cysts of the lung or of the pancreas, cavernous angiomas of the liver, angiomas of the epididymis, and cutaneous angiomas.…”

Section: Discussionmentioning

confidence: 99%

Importance of the Perineural Spaces in Nerve Blocking

Moore¹,

Hain²,

Ward³

et al. 1954

JAMA

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Physicians who inject nerve blocking agents, either to produce local anesthesia or to alleviate pain, are aware of the possible complications associated with these procedures. However, few seem to be cognizant of the potential significance of the relationship of the perineural spaces to the subarachnoid space and the spinal cord as the determining factor in the development of some of the more serious complications. This relationship was bluntly called to our attention by recent experiences after the use of Efocaine (a solution of 1% procaine, 0.25% procaine hydrochloride, and 5% butyl-p-aminobenzoate in a solvent composed of 2% polyethylene glycol 300, 78% propylene glycol, and water). It was originally advertised as a safe, long-acting (two to four weeks' duration), local anesthetic mixture unassociated with encapsulation, abscesses, foreign body reactions, tissue sloughs, or other such adverse effects encountered after the use of oil solvents.1 Early published reports seemed to support the claim of its manufacturer that its prolonged action was effected by the precipitation of procaine crystals in the body tissues.-However, later pub¬ lished reports indicated that the long action was actually due to tissue damage, particularly to nervous tissue, from the propylene and polyethylene glycol that are the principal solvents in the Efocaine mixture. These pub¬ lications reported sloughs, cellulitis, transverse myelitis, and deaths.3In reviewing two deaths and 11 cases of transverse myelitis following the use of Efocaine, it was noted ;i0 that all of the patients received paravertebral injections of the peripheral somatic nerves except one, who re¬ ceived a lumbar sympathetic block. The maximum dis¬ tance of the injection site from the intervertebral foramen was 8 cm., by actual postmortem measurement. Four of these patients had percutaneous injections-in two the intercostal nerves were blocked, and in one the lum¬ bar sympathetic nerves were injected. In the remaining nine patients, the intercostal nerves were injected intraneurally under direct vision for the relief of postopera¬ tive pain prior to closure of thoracotomies. In most instances, the physician blamed himself for having in¬ advertently injected the anesthetic agent into the subarachnoid space through unusually long cuffs of dura. This is exemplified by the following statement from a recently published paper by Brittingham, Berlin, and Wolff *: "An almost unavoidable hazard of paravertebral nerve block is inadvertent injection of the agent used into the subarachnoid space. This probably occurs be¬ cause of an outward prolongation of the subarachnoid space or in other instances because of movement of the patient and tearing of the tissues after the needle is in place." In spite of this possibility, it is unlikely that durai cuffs extend 6 to 8 cm. past the intervertebral foramina; thus, direct injection into the subarachnoid space by this route is doubtful.5If long durai cuffs are not the route of entrance into the spinal subarachnoid space, what other rou...

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Multiple Hereditary Hemangioblastomas of the Nervous System

Cited by 28 publications

References 14 publications

Comparative Analysis of Spinal Hemangioblastomas in Sporadic Disease and Von Hippel-Lindau Syndrome

Comparative Analysis of Spinal Hemangioblastomas in Sporadic Disease and Von Hippel-Lindau Syndrome

Familial von Hippel-Lindau's Disease

Importance of the Perineural Spaces in Nerve Blocking

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