Syringomyelia (Morvan's Disease)

Longitudinal cavities in the spinal cord, most often in the cervical region, which may extend for multiple spinal levels. The cavities are lined by dense, gliogenous tissue and may be associated with SPINAL CORD NEOPLASMS; spinal cord traumatic injuries; and vascular malformations. Syringomyelia is marked clinically by pain and PARESTHESIA, muscular atrophy of the hands, and analgesia with thermoanesthesia of the hands and arms, but with the tactile sense preserved (sensory dissociation). Lower extremity spasticity and incontinence may also develop. (From Adams et al., Principles of Neurology, 6th ed, p1269)

Also Known As:

Networked: 540 relevant articles (33 outcomes, 39 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Scoliosis
2.	Hydrocephalus (Hydrocephaly)
3.	Pain (Aches)
4.	Encephalocele (Encephalocele, Frontal)
5.	Meningitis

Experts

1.	Tubbs, R Shane: 9 articles (12/2013 - 04/2003)
2.	Qiu, Yong: 8 articles (05/2015 - 01/2006)
3.	Oakes, W Jerry: 8 articles (12/2013 - 04/2003)
4.	Stoodley, Marcus A: 7 articles (08/2013 - 06/2003)
5.	Zhu, Zezhang: 6 articles (05/2015 - 01/2006)
6.	Heiss, John D: 6 articles (11/2012 - 02/2008)
7.	Tu, Jian: 4 articles (01/2015 - 06/2003)
8.	Wellons, John C: 4 articles (10/2013 - 04/2003)
9.	Bilston, Lynne E: 4 articles (08/2013 - 04/2010)
10.	Cheng, Shaokoon: 4 articles (08/2013 - 04/2012)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Syringomyelia:

1.	Familial spinal arachnoiditisIBA 07/01/2010 - "Three of 6 patients with syringomyelia due to spinal arachnoiditis improved. " 08/01/2000 - "Familial spinal arachnoiditis with secondary syringomyelia: clinical studies and MRI findings." 01/01/2014 - "Syringomyelia associated with spinal arachnoiditis treated by partial arachnoid dissection and syrinx-far distal subarachnoid shunt." 04/01/2012 - "Spinal arachnoiditis and spinal syringomyelia are rare but important chronic complications after SAH." 04/01/2012 - "We describe two patients with syringomyelia associated with chronic spinal arachnoiditis following SAH. "
2.	IronIBA 11/01/1989 - "During a 12 month period of operation of a 0.3 Tesla MRI iron cored resistive scanner 74 cases of syringomyelia were diagnosed on clinical, radiological and/or surgical grounds. " 01/01/2013 - "We report a patient who developed superficial siderosis following neurosurgery for syringomyelia and who had an improvement in his hearing and mobility following treatment with a new iron chelation therapy that can penetrate the blood-brain barrier. "
3.	Penicillins (Penicillin)FDA Link 06/01/2011 - "A case of syphilitic spinal meningitis presenting with syringomyelia, and effectively treated with penicillin has been described."
4.	MetrizamideFDA Link 01/01/1987 - "The objectives of this study were to compare the accuracy of conventional metrizamide myelography with CT metrizamide myelography and to study indirectly the hydrodynamics of CSF flow in syringomyelia by comparing the sequential enhancement patterns of the spinal cords and cord cavities in the different groups of patients. " 09/01/1984 - "During the period of this study, metrizamide myelography in conjunction with early and delayed computerized tomography scanning replaced all other diagnostic procedures in patients with syringomyelia. " 10/01/1992 - "On the basis of metrizamide CT and cranial MRI, a diagnosis of syringomyelia with Chiari malformation (type I) was made. " 12/01/1987 - "Syringomyelia was then in evidence as seen by myelography and delayed metrizamide computed tomography scanning. " 08/01/1987 - "Post metrizamide CT in the diagnosis of syringomyelia."
5.	KaolinIBA 01/15/2005 - "Origin of macrophages in a kaolin-induced model of rat syringomyelia: a study using radiation bone marrow chimeras." 06/01/1980 - "The influence of ventricular pressure changes on syrinx pressure was investigated in this study of experimental canine kaolin-induced syringomyelia. " 12/01/2012 - "A kaolin-induced model of canalicular syringomyelia was used to investigate BSCB permeability and AQP4 expression in 27 adult male Sprague-Dawley rats. " 10/01/2011 - "Syringomyelia was induced in the experimental group by kaolin injection into the cisterna magna. " 04/18/2007 - "The experimental syringomyelia models of rabbits were established by intra-cisternal injection of Kaolin. "
6.	SASIBA 11/01/2012 - "These findings are similar to the results of the authors' previous studies that examined the pathophysiology of syringomyelia associated with obstruction of the SAS at the foramen magnum in the Chiari Type I malformation and indicate that a common mechanism, rather than different, separate mechanisms, underlies syrinx formation in these two entities. " 12/01/2005 - "The presented in vitro study of spinal cord wall motion, and complex unsteady pressure and flow environment within the syrinx and SAS, provides insight into the complex biomechanical forces present in syringomyelia." 11/01/2012 - "The authors hypothesized that in primary spinal syringomyelia, a subarachnoid block effectively shortens the length of the spinal subarachnoid space (SAS), reducing compliance and the ability of the spinal theca to dampen the subarachnoid CSF pressure waves produced by brain expansion during cardiac systole. " 04/01/2010 - "The mechanisms of syringomyelia have long puzzled neurosurgeons and researchers alike due to difficulties in identifying the driving forces behind fluid flow into a syrinx, apparently against a pressure gradient between the spinal cord and the subarachnoid space (SAS). " 12/01/2005 - "In vivo magnetic resonance imaging (MRI) measurements of subarachnoid (SAS) geometry and cerebrospinal fluid velocity were acquired in a patient with syringomyelia and used to aid in the in vitro model design and experiment. "
7.	tebufenozide (Mimic)IBA 01/01/2015 - "Syringomyelia is a potentially serious neurologic condition with symptoms that can mimic other neuromuscular disorders. " 01/01/2015 - "Clinical presentation of syringomyelia can mimic a variety of neuromuscular disorders. " 07/01/2009 - "A treatable mimic of Chiari malformation with syringomyelia." 04/01/2010 - "Syringomyelia can mimic intramedullary tumors clinically and radiologically. " 09/01/2000 - "Syringomyelia may clinically mimic ulnar neuropathy at the elbow."
8.	Substance PIBA 06/01/1996 - "In nine of 10 patients with syringomyelia, there was a substantial increase in substance P immunoreactivity in the first, second, third, and fifth laminae below the level of the lesion. " 06/01/1996 - "Distribution of substance P in the spinal cord of patients with syringomyelia." 06/01/1996 - "The authors conclude that syringomyelia can be associated with abnormalities in spinal cord levels of substance P, which may affect the modulation and perception of pain." 05/01/2013 - "Concentrations of substance P were significantly higher in dogs with asymmetrical syringomyelia or dorsal horn involvement, whereas interleukin-6 concentrations were not significantly different between groups. " 08/01/2012 - "Distribution of substance P and calcitonin gene-related peptide in the spinal cord of Cavalier King Charles Spaniels affected by symptomatic syringomyelia."
9.	HormonesIBA 12/01/2013 - "If a trial of hormone replacement is attempted, patients should be followed closely for progression of syringomyelia or the development of symptoms." 01/01/2014 - "Syringomyelia associated with inappropriate antidiuretic hormone secretion."
10.	Calcitonin Gene-Related PeptideIBA 08/01/2012 - "Distribution of substance P and calcitonin gene-related peptide in the spinal cord of Cavalier King Charles Spaniels affected by symptomatic syringomyelia." 08/01/2012 - "In this study we compared expression of two pain-related neuropeptides: substance P (SP) and calcitonin gene related peptide (CGRP), in the spinal cord dorsal horn of normal dogs with that in CKCS with and without clinical signs of syringomyelia. "

Therapies and Procedures

1.	Decompression 04/01/2009 - "Our data revealed that for both groups of patients, with or without associated syringomyelia, the predominant improvement in central conduction in most cases occurred during the period of bony decompression without significant additional improvement after the duraplasty procedure." 10/01/2007 - "The osteo-dural decompression of the cerebellar tonsils at the cranio-cervical junction is generally considered the most effective treatment for syringomyelia-Chiari I complex. " 05/01/2010 - "His postoperative neurological status had improved remarkably, with imaging showing good realignment of the occipito-atlanto-axial complex with comfortable decompression of the cervico-medulla junction and relief of syringomyelia. " 09/01/2006 - "In all but one patient, syringomyelia improved significantly after cranio-vertebral decompression. " 01/01/1998 - "These results suggest that foramen magnum decompression combined with transverse microincisions of the outer layer of the dura 1) is an effective and safe treatment option for syringomyelia and Chiari I malformation, 2) corrects the circulatory disturbances of cerebrospinal fluid dynamic, 3) leads to a decrease in size of the syrinx and to a significant improvement in neurological signs and symptoms, 4) avoids complications of intradural approaches and syringosubarachnoid shunting."
2.	Ventriculostomy 07/01/2011 - "In select cases, terminal ventriculostomy may help preserve neurological function in the face of otherwise progressive syringomyelia." 07/01/2011 - "Terminal ventriculostomy as an adjuvant treatment of complex syringomyelia: a case report and review of the literature." 04/01/2011 - "It can be successfully treated by endoscopic third ventriculostomy (ETV), possibly improving related CIM and syringomyelia. " 12/01/2009 - "Of 240 hydrocephalic patients treated in our institution with endoscopic third ventriculostomy, a subgroup of 10 cases with closure of the fourth ventricular outlets without associated Chiari malformation and syringomyelia was selected. " 01/01/2002 - "Successful endoscopic third ventriculostomy in noncommunicating thoracic syringomyelia."
3.	Laminectomy 02/18/2008 - "These data, compared with the literature, allow us to conclude that suboccipital craniectomy and Cl laminectomy (possibly integrated by dural delamination) is an effective and safe treatment for symptomatic children with Chiari I malformation and syringomyelia. " 08/01/2007 - "This study aims to analyze the effect of laminectomies performed in the course of treatment of syringomyelia. " 01/01/2012 - "A recent MRI demonstrated worsening of cervical xyphosis after several laminectomies and residual syringomyelia besides syringopleural shunt. " 01/01/2011 - "A 47-year-old woman underwent decompressive suboccipital craniectomy and C1 laminectomy with duroplasty in the prone position for Chiari malformation type I and syringomyelia. " 09/01/2010 - "Laminectomy for syringomyelia, with reconstruction of the spinal CSF channels alone, resulted in a reduction of syrinx size by 6 months, with half of all patients experiencing stabilization of symptoms in the long term. "
4.	Surgical Decompression 06/16/2013 - "So surgical decompression, enlarging medullary canal prevents rubbings and contacts between the bone-ligament structures of the spine towards spinal cord and subarachnoid space therefore syringomyelia. " 07/01/2002 - "All patients were treated with surgical decompression of the Chiari malformation with or without drainage of the syringomyelia. " 02/01/2007 - "In 1 patient with Chiari malformation with syringomyelia, we made a 2.5 cm incision under the endoscope for the surgical decompression of the foramen magnum. " 12/01/1995 - "Surgical procedures included posterior brain stem decompression in 12 patients, plugging of the obex in 7, and placement of syringosubarachnoid shunts in 7, a syringopleural shunt in 1, and fourth ventricular stents in 2. In the 12 patients with syringomyelia, symptoms from brain stem compression dramatically improved with surgical decompression (P < 0.025), whereas symptoms from syringomyelia less dramatically improved or stabilized. " 01/01/2009 - "The most effective therapy for patients with Chiari type I malformation/syringomyelia is surgical decompression of the foramen magnum, however there are non-surgical therapy to relieve neuropathic pain: either pharmacological and non-pharmacological. "
5.	Craniotomy 03/01/2015 - "In contrast to published data, the authors describe the case of a 32-year-old woman who underwent surgery for CM associated with extensive cervicothoracic syringomyelia and whose intraoperative somatosensory evoked potentials (SSEPs) did not notably improve after craniotomy or following durotomy; rather, they only improved after opening of the fourth ventricle and restoration of CSF flow through the foramen of Magendie. " 07/01/2004 - "We describe a simple method of expansive suboccipital cranioplastic craniotomy using a free bone flap and report satisfactory clinical results in 16 patients with syringomyelia associated with Chiari I malformation. " 03/01/1985 - "We describe a patient with a strongly positive family history of this disease who, at age 28, underwent craniotomy with removal of a cystic cerebellar hemangioblastoma; at age 48, he developed syringomyelia of the spinal cord, became quadriplegic, and had a progressive downhill course. " 04/15/2005 - "Suboccipital craniotomy and laminectomy of the first cervical vertebra were performed, and substantial improvement in the scoliosis and syringomyelia was observed 3 months after surgery. " 11/01/1998 - "Procedures included 2 brain biopsies, 5 craniotomies for tumor, 2 thoracic laminectomies for syringomyelia, and placement of a reservoir into a cystic brainstem tumor. "