Spontaneous spinal cerebrospinal fluid leaks and minor skeletal features of Marfan syndrome: a microfibrillopathy

2002 ◽  
Vol 96 (3) ◽  
pp. 483-489 ◽  
Author(s):  
Iris Schrijver ◽  
Wouter I. Schievink ◽  
Maurice Godfrey ◽  
Fredric B. Meyer ◽  
Uta Francke
Keyword(s):  
Cerebrospinal Fluid ◽  
Connective Tissue ◽  
Marfan Syndrome ◽  
Metabolic Labeling ◽  
Cultured Fibroblasts ◽  
Content Type ◽  
Skeletal Abnormalities ◽  
Fibrillin 1 ◽  
Fine Print ◽  
Csf Leaks

Object. Spontaneous spinal cerebrospinal fluid (CSF) leaks are increasingly recognized as a cause of postural headaches. The authors examined a group of patients suffering from spontaneous spinal CSF leaks who also had minor skeletal features of Marfan syndrome for abnormalities of fibrillin-containing microfibrils. Methods. Patients with spontaneous CSF leaks were evaluated for the clinical characteristics of connective tissue disorders. Skin biopsies were obtained in three patients with skeletal manifestations that constitute part of the Marfan syndrome phenotype. Cultured fibroblasts were studied for fibrillin-1 synthesis and incorporation into the extracellular matrix (ECM) by performing quantitative metabolic labeling and immunohistochemical analysis. Among 20 consecutive patients found to have spinal CSF leaks, four (20%) exhibited minor skeletal features of Marfan syndrome, but lacked any ocular or cardiovascular abnormalities. The mean age of these patients (30 years) was lower than that of the 16 patients without skeletal abnormalities (44 years; p = 0.01). Abnormalities in fibrillin1 metabolism and immunostaining were detected in all three patients with the skeletal abnormalities who underwent examination, but not in a control patient without these skeletal manifestations. Conclusions. Twenty percent of patients who experience spontaneous spinal CSF leaks have minor skeletal features of Marfan syndrome. The authors demonstrated abnormalities in fibrillin-1 protein deposition in all patients examined, but only one person was found to have a fibrillin-1 abnormality typically found in classic Marfan syndrome. The results indicate that there is a heterogeneous involvement of other components of ECM microfibrils at the basis of this cerebrospinal manifestation. In addition, the authors identified a connective-tissue etiological factor in a group of disorders not previously classified as such.

Subcutaneous reaction to silicone in ventriculoperitoneal shunts

1974 ◽  
Vol 41 (3) ◽  
pp. 367-371 ◽  
Author(s):  
Oscar Sugar ◽  
Orville T. Bailey
Keyword(s):  
Cerebrospinal Fluid ◽  
Connective Tissue ◽  
Fibrous Connective Tissue ◽  
Subcutaneous Implantation ◽  
Content Type ◽  
Ventriculoperitoneal Shunts ◽  
Silicone Tubing ◽  
Connective Tissue Sheath ◽  
Fine Print

✓Silicone tubing (Silastic) used for ventriculoperitoneal shunts induces a fibrous connective tissue sheath around the tubing in children and adults. Two children examined 8 and 3 years after subcutaneous implantation showed a complete tube of dense fibrous connective tissue around the silicone tubing. The reaction was entirely quiescent. These tubes of connective tissue were apparently capable of conveying cerebrospinal fluid for some months after the silicone tubing was disconnected from the pump or pulled out of the abdomen.

Surgical treatment of spontaneous spinal cerebrospinal fluid leaks

1998 ◽  
Vol 88 (2) ◽  
pp. 243-246 ◽  
Author(s):  
Wouter I. Schievink ◽  
Vittorio M. Morreale ◽  
John L. D. Atkinson ◽  
Fredric B. Meyer ◽  
David G. Piepgras ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Surgical Treatment ◽  
Intracranial Hypotension ◽  
Csf Leak ◽  
Preoperative Imaging ◽  
Content Type ◽  
Surgical Exploration ◽  
Fine Print ◽  
Csf Leaks

Object. Spontaneous spinal cerebrospinal fluid (CSF) leaks are an increasingly recognized cause of intracranial hypotension and may require neurosurgical intervention. In the present report the authors review their experience with the surgical management of spontaneous spinal CSF leaks. Methods. Between 1992 and 1997, 10 patients with spontaneous spinal CSF leaks and intracranial hypotension were treated surgically. The mean age of the seven women and three men was 42.3 years (range 22–61 years). Preoperative imaging showed a single meningeal diverticulum in two patients, a complex of diverticula in one patient, and a focal CSF leak alone in seven patients. Surgical exploration in these seven patients demonstrated meningeal diverticula in one patient; no clear source of CSF leakage could be identified in the remaining six patients. Treatment consisted of ligation of the diverticula or packing of the epidural space with muscle or Gelfoam. Multiple simultaneous spinal CSF leaks were identified in three patients. Conclusions. All patients experienced complete relief of their headaches postoperatively. There has been no recurrence of symptoms in any of the patients during a mean follow-up period of 19 months (range 3–58 months; 16 person-years of cumulative follow up). Complications consisted of transient intracranial hypertension in one patient and leg numbness in another patient. Although the disease is often self-limiting, surgical treatment has an important role in the management of spontaneous spinal CSF leaks. Surgery is effective in eliminating the headaches and the morbidity is generally low. Surgical exploration for a focal CSF leak, as demonstrated on radiographic studies, usually does not reveal a clear source of the leak. Some patients may have multiple simultaneous CSF leaks.

Ultrastructural morphology of the olfactory pathway for cerebrospinal fluid drainage in the rabbit

1986 ◽  
Vol 64 (3) ◽  
pp. 466-473 ◽  
Author(s):  
Stephanie S. Erlich ◽  
J. Gordon McComb ◽  
Shigeyo Hyman ◽  
Martin H. Weiss
Keyword(s):  
Cerebrospinal Fluid ◽  
Connective Tissue ◽  
Electron Microscopic Examination ◽  
Olfactory Pathway ◽  
Electron Microscopic ◽  
En Bloc ◽  
Major Pathway ◽  
Content Type ◽  
Csf Drainage ◽  
Fine Print

✓ Previous physiological studies indicate that the olfactory region serves as a major pathway for cerebrospinal fluid (CSF) drainage into the lymphatic system. The present study was undertaken to determine the ultrastructural characteristics of this egress route. New Zealand White rabbits received a single bolus injection of the tracer ferritin (MW 400,000) into both lateral ventricles in such a manner as not to raise the intraventricular pressure above the normal level. The animals were sacrificed via intracardiac perfusion of fixative between less than 12 minutes and 4 hours following injection. The cribriform region was removed en bloc, decalcified, sectioned coronally, and prepared for light and electron microscopic examination. The arachnoid, dura, and periosteum surrounding the fila olfactoria passing through the cribriform plate merge together and form the perineurium, which consists of multiple layers of loosely overlapping cells with widely separated junctions and few vesicles. The perineurium surrounding the olfactory filaments at the superficial submucosal level is only one cell thick. The subarachnoid space freely communicates with the perineural space surrounding each filament. No morphological barrier between the perineural space and the loose submucosal connective tissue was identified. Whether or not the perineurium was multi- or singlelayered, ferritin was noted in abundance between the loosely overlapping perineural cells and in the submucosal connective tissue. The distribution of ferritin at 12 minutes was similar to that at 4 hours; however, the quantity of ferritin was increased at 4 hours. These results indicate that no significant barrier to CSF drainage is present at the rabbit cribriform region and that CSF reaches the submucosal region rapidly via open pathways.

A physiological sealant for cerebrospinal fluid leaks

1977 ◽  
Vol 46 (2) ◽  
pp. 215-219 ◽  
Author(s):  
Gerald D. Silverberg ◽  
Christina B. Harbury ◽  
Edward Rubenstein
Keyword(s):  
Cerebrospinal Fluid ◽  
Histological Examination ◽  
Content Type ◽  
Cerebrospinal Fluid Leaks ◽  
The Brain ◽  
Fine Print ◽  
Csf Leaks

✓ A combination of concentrated platelets, thrombin, and fibrinogen was used to adhere a pericranial graft to surgically produced cerebrospinal fluid (CSF) fistulas in dogs. This sealant successfully stopped leakage of CSF in all fistulas produced in both acute and chronic preparations. All control animals leaked CSF acutely. In chronic control animals the CSF leaks sealed spontaneously but the grafts were not well incorporated. Histological examination of the grafts and underlying brain showed no injury to the brain or meningeal vessel from exposure to the platelet glue. Good fibrous union of the grafts to the dura was confirmed.

Leptomeningeal cysts in congenital ectopia lentis

1973 ◽  
Vol 38 (5) ◽  
pp. 650-654 ◽  
Author(s):  
Bryce Weir
Keyword(s):  
Connective Tissue ◽  
Marfan Syndrome ◽  
Genetic Disorder ◽  
Ectopia Lentis ◽  
Content Type ◽  
Extradural Cyst ◽  
Common Pathogenesis ◽  
Intrathoracic Meningocele ◽  
Subarachnoid Cyst ◽  
Fine Print

✓ A case of ectopia lentis (Marfan syndrome) with a cerebral subarachnoid cyst, a spinal extradural cyst connected to a lateral intrathoracic meningocele, and perineural cysts is presented. The association of these leptomeningeal cysts in this genetic disorder of connective tissue suggests a common pathogenesis.

Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension

1996 ◽  
Vol 84 (4) ◽  
pp. 598-605 ◽  
Author(s):  
Wouter I. Schievink ◽  
Fredric B. Meyer ◽  
John L. D. Atkinson ◽  
Bahram Mokri
Keyword(s):  
Cerebrospinal Fluid ◽  
Thoracic Spine ◽  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
Csf Leak ◽  
Cervicothoracic Junction ◽  
Ct Myelography ◽  
Content Type ◽  
Fine Print ◽  
Csf Leaks

✓ Spinal cerebrospinal fluid (CSF) leaks are often implicated as the cause of the syndrome of spontaneous intracranial hypotension, but they have rarely been demonstrated radiographically or surgically. The authors reviewed their experience with documented cases of spinal CSF leaks of spontaneous onset in 11 patients including their surgical observations in four of the patients. The mean age of the six women and five men included in the study was 38 years (range 22–51 years). All patients presented with a postural headache; however, most had additional symptoms, including nausea, emesis, sixth cranial-nerve paresis, or local back pain at the level of the CSF leak. All patients underwent indium-111 radionucleotide cisternography or computerized tomographic (CT) myelography. The location of the spontaneous CSF leak was in the cervical spine in two patients, the cervicothoracic junction in three patients, the thoracic spine in five patients, and the lumbar spine in one patient. The false negative rate for radionucleotide cisternography was high (30%). Subdural fluid collections, meningeal enhancement, and downward displacement of the cerebellum, resembling a Chiari I malformation, were commonly found on cranial imaging studies. In most patients, the symptoms resolved in response to supportive measures or an epidural blood patch. Leaking meningeal diverticula were found to be the cause of the CSF leak in four patients who underwent surgery. In three patients these diverticula could be ligated with good result but in one patient an extensive complex of meningeal diverticula was found to be inoperable. Two patients had an unusual body habitus and joint hypermobility, and two other patients had suffered a spontaneous retinal detachment at a young age. In conclusion, spontaneous spinal CSF leaks are uncommon, but they are increasingly recognized as a cause of spontaneous intracranial hypotension. Most spinal CSF leaks are located at the cervicothoracic junction or in the thoracic spine, and they may be associated with meningeal diverticula. The radiographic study of choice is CT myelography. The disease is usually self-limiting, but in selected cases our experience with surgical ligation of leaking meningeal diverticula has been satisfactory. An underlying connective tissue disorder may be present in some patients with a spontaneous spinal CSF leak.

Recurrent intracranial solitary fibrous tumor with cerebrospinal fluid dissemination

2004 ◽  
Vol 101 (6) ◽  
pp. 1045-1048 ◽  
Author(s):  
Katsuyoshi Miyashita ◽  
Yutaka Hayashi ◽  
Hironori Fujisawa ◽  
Mitsuhiro Hasegawa ◽  
Junkoh Yamashita
Keyword(s):  
Cerebrospinal Fluid ◽  
Malignant Transformation ◽  
Solitary Fibrous Tumor ◽  
S100 Protein ◽  
Spinal Tumors ◽  
Content Type ◽  
Csf Dissemination ◽  
Fibrous Tumor ◽  
Fine Print ◽  
Mib 1

✓ Solitary fibrous tumor (SFT) is a benign and rare neoplasm. To date, only 37 patients with intracranial SFTs have been reported. Although a number of the tumors were recurrent and some later underwent malignant transformation, none of these lesions progressed to cerebrospinal fluid (CSF) dissemination. In this paper the authors report a case of SFT in which the lesion recurred several times and ultimately was disseminated by the CSF. The patient was a 63-year-old woman with multiple intracranial and spinal tumors. Fifteen years before this presentation, at the age of 48 she had been hospitalized for resection of a falcotentorial tumor. During the ensuing 15 years she underwent multiple surgeries and sessions of radiation therapy for recurrent lesions. The exclusive location of her tumors in the subarachnoid space at the end of this 15-year period indicate CSF dissemination of the tumor. The tumor that was resected when the patient was 48 years old and the latest resected lesion were analyzed by performing immunohistological CD34, epithelial membrane antigen, vimentin, S100 protein, and reticulin staining, and determining the MIB-1 labeling index (LI). Most of the results were identical, and both tumors were diagnosed as SFT according to a staining pattern that showed a strong and diffuse positive reaction for CD34. Nevertheless, the authors noted that the MIB-1 LI increased from less than 1% in the original tumor to 13% in the latest tumor. The increased proliferation of MIB-1 indicates that the malignant transformation could have occurred during tumor recurrence with CSF dissemination.

Evaluation of endorphin content in the CSF of patients with trigeminal neuralgia before and after Gasserian ganglion thermocoagulation

1981 ◽  
Vol 55 (6) ◽  
pp. 935-937 ◽  
Author(s):  
Giuseppe Salar ◽  
Salvatore Mingrino ◽  
Marco Trabucchi ◽  
Angelo Bosio ◽  
Carlo Semenza
Keyword(s):  
Cerebrospinal Fluid ◽  
Trigeminal Neuralgia ◽  
Control Group ◽  
Gasserian Ganglion ◽  
Content Type ◽  
Group Of Seven ◽  
Idiopathic Trigeminal Neuralgia ◽  
Significant Difference ◽  
Before And After ◽  
Fine Print

✓ The β-endorphin content in cerebrospinal fluid (CSF) was evaluated in 10 patients with idiopathic trigeminal neuralgia during medical treatment (with or without carbamazepine) and after selective thermocoagulation of the Gasserian ganglion. These values were compared with those obtained in a control group of seven patients without pain problems. No statistically significant difference was found between patients suffering from trigeminal neuralgia and those without pain. Furthermore, neither pharmacological treatment nor surgery changed CSF endorphin values. It is concluded that there is no pathogenetic relationship between trigeminal neuralgia and endorphins.

Acquired Chiari I malformation secondary to spontaneous spinal cerebrospinal fluid leakage and chronic intracranial hypotension syndrome in seven cases

1998 ◽  
Vol 88 (2) ◽  
pp. 237-242 ◽  
Author(s):  
John L. D. Atkinson ◽  
Brian G. Weinshenker ◽  
Gary M. Miller ◽  
David G. Piepgras ◽  
Bahram Mokri
Keyword(s):  
Cerebrospinal Fluid ◽  
Intracranial Hypotension ◽  
Chiari I Malformation ◽  
Csf Leak ◽  
Cerebrospinal Fluid Leakage ◽  
Content Type ◽  
Intracranial Hypotension Syndrome ◽  
Csf Leakage ◽  
Chiari I ◽  
Fine Print

Object. Spontaneous spinal cerebrospinal fluid (CSF) leakage with development of the intracranial hypotension syndrome and acquired Chiari I malformation due to lumbar spinal CSF diversion procedures have both been well described. However, concomitant presentation of both syndromes has rarely been reported. The object of this paper is to present data in seven cases in which both syndromes were present. Three illustrative cases are reported in detail. Methods. The authors describe seven symptomatic cases of spontaneous spinal CSF leakage with chronic intracranial hypotension syndrome in which magnetic resonance (MR) images depicted dural enhancement, brain sagging, loss of CSF cisterns, and acquired Chiari I malformation. Conclusions. This subtype of intracranial hypotension syndrome probably results from chronic spinal drainage of CSF or high-flow CSF shunting and subsequent loss of brain buoyancy that results in brain settling and herniation of hindbrain structures through the foramen magnum. Of 35 cases of spontaneous spinal CSF leakage identified in the authors' practice over the last decade, MR imaging evidence of acquired Chiari I malformation has been shown in seven. Not to be confused with idiopathic Chiari I malformation, ideal therapy requires recognition of the syndrome and treatment directed to the site of the spinal CSF leak.

Effects of indomethacin and prostacyclin on isolated human pial arteries contracted by CSF from patients with aneurysmal SAH

1981 ◽  
Vol 55 (6) ◽  
pp. 877-883 ◽  
Author(s):  
Lennart Brandt ◽  
Bengt Ljunggren ◽  
Karl-Erik Andersson ◽  
Bengt Hindfelt ◽  
Tore Uski
Keyword(s):  
Cerebrospinal Fluid ◽  
Arachidonic Acid ◽  
Vessel Wall ◽  
Cerebral Arteries ◽  
Pial Arteries ◽  
Content Type ◽  
Cerebral Vasoconstriction ◽  
Arachidonic Acid Metabolites ◽  
Acid Metabolites ◽  
Fine Print

✓ In small human cerebral arteries preincubated with indomethacin, contractions induced by cerebrospinal fluid (CSF), from patients with subarachnoid hemorrhage were markedly increased. Also contractions induced by noradrenaline, but not 5-hydroxytryptamine, were augmented. Prostacyclin and its metabolite 6-keto-prostaglandin (PG)E1 reversed the contractions induced by CSF, as well as by noradrenaline, 5-hydroxytryptamine, and PGF2α. The findings suggest that these substances are able to counteract the influence of vasoconstrictor material in hemorrhagic CSF. If the capacity to synthesize these “protective” arachidonic acid metabolites is reduced, the resulting imbalance between contractile and relaxant forces acting on the vessel wall may lead to sustained cerebral vasoconstriction.

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