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PubMed1995

Pathological basis of spinal cord cavitation in syringomyelia: analysis of 105 autopsy cases.

This report summarizes neuropathological, clinical, and general autopsy findings in 105 individuals with nonneoplastic syringomyelia. On the basis of detailed histological findings, three types of cavities were distinguished: 1) dilations of the central canal that communicated directly with the fourth ventricle (47 cases); 2) noncommunicating (isolated) dilations of the central canal that arose below a syrinx-free segment of spinal cord (23 cases); and 3) extracanalicular syrinxes that originated in the spinal cord parenchyma and did not communicate with the central canal (35 cases). The incidence of communicating syrinxes in this study reflects an autopsy bias of morbid conditions such as severe birth defects. Communicating central canal syrinxes were found in association with hydrocephalus. The cavities were lined wholly or partially by ependyma and their overall length was influenced by age-related stenosis of the central canal. Non-communicating central canal syrinxes arose at a variable distance below the fourth ventricle and were associated with disorders that presumably affect cerebrospinal fluid dynamics in the spinal subarachnoid space, such as the Chiari I malformation, basilar impression, and arachnoiditis. These cavities were usually defined rostrally and caudally by stenosis of the central canal and were much more likely than communicating syrinxes to dissect paracentrally into the parenchymal tissues. The paracentral dissections of the central canal syrinxes occurred preferentially into the posterolateral quadrant of the spinal cord. Extracanalicular (parenchymal) syrinxes were found typically in the watershed area of the spinal cord and were associated with conditions that injure spinal cord tissue (for example, trauma, infarction, and hemorrhage). A distinguishing feature of this type of cavitation was its frequent association with myelomalacia. Extracanalicular syrinxes and the paracentral dissections of central canal syrinxes were lined by glial or fibroglial tissue, ruptured frequently into the spinal subarachnoid space, and were characterized by the presence of central chromatolysis, neuronophagia, and Wallerian degeneration. Some lesions extended rostrally into the medulla or pons (syringobulbia). Although clinical information was incomplete, simple dilations of the central canal tended to produce nonspecific neurological findings such as spastic paraparesis, whereas deficits associated with extracanalicular syrinxes and the paracentral dissections of central canal syrinxes included segmental signs that were referable to affected nuclei and tracts. It is concluded that syringomyelia has several distinct cavitary patterns with different mechanisms of pathogenesis that probably determine the clinical features of the condition.

Adolescent↗
PubMed1996

Comparison of syringopleural and syringosubarachnoid shunting in the treatment of syringomyelia in children.

Case records from the Montreal Children's Hospital containing the diagnosis of shunted syringomyelia were retrospectively reviewed. From 1984 to 1994, 31 patients had their syrinx treated by either syringopleural (19 cases, Group A) or syringosubarachnoid (13 cases, Group B) shunting. One patient was included in both groups. Associated diagnoses included: in Group A, two cases of Chiari I and 14 of Chiari II malformations, 14 cases of shunted hydrocephalus, 13 cases of spina bifida aperta, and three cases of spina bifida occulta; Group B, four cases of Chiari I and two of Chiari II malformations, four cases of shunted hydrocephalus, two cases of spina bifida aperta, and five cases of spina bifida occulta. Eight Group A and six Group B patients had undergone prior posterior fossa decompression. Motor deficits predominated in both groups and arachnoiditis was a uniform operative finding. Neurological follow-up examinations showed 11 Group A patients improved and eight stabilized, whereas on magnetic resonance imaging, 12 cavities appeared to have collapsed, five were markedly reduced, and one had increased. One patient underwent reoperation for pleural effusions and one for shunt displacement. In Group B, one patient improved, eight stabilized, three worsened neurologically, and one was lost to follow-up review. Radiologically, one cavity appeared to have collapsed, six were significantly reduced, two were unchanged, and three had enlarged. The authors conclude that syringopleural shunting is a valuable option for controlling syringomyelia in patients without Chiari malformation or in patients who have previously undergone a craniovertebral decompression or are otherwise asymptomatic from their Chiari malformation.

Adolescent↗
PubMed1996

Distribution of substance P in the spinal cord of patients with syringomyelia.

The distribution of substance P, a putative neurotransmitter and pain-related peptide, was studied using the peroxidase-antiperoxidase immunohistochemical method in the spinal cords obtained from autopsy of 10 patients with syringo-myelia and 10 age- and sex-matched, neurologically normal individuals. Substance P immunoreactivity was present in axons and in terminal-like processes in close apposition to neurons in the first, second, and third laminae of the dorsal horn. Smaller amounts of peroxidase-positive staining were found in the fifth lamina of the dorsal horn, the intermediolateral nucleus, the intermediomedial nucleus, and the ventral horn. 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. A marked reduction or absence of staining was present in segments of the spinal cord occupied by the syrinx. Central cavities produced bilateral abnormalities, whereas eccentric cavities produced changes that were ipsilateral to the lesion. No alterations in staining were found in the spinal cord of an asymptomatic patient with a small central syrinx. 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.

Adult↗
PubMed1996

Management and outcome of posttraumatic syringomyelia.

Traumatic paraplegia is the most common cause of nonhindbrain-related syringomyelia. Fifty-seven patients with a mean age of 34.3 years at presentation were treated at the Midland Centre for Neurosurgery and Neurology between 1973 and 1993. A variety of treatment strategies have been used over the years, including syringosubarachnoid and syringopleural shunts, spinal cord transection, and pedicled omental graft transposition. More recently decompressive laminectomy, subarachnoid space reconstruction and formation of surgical meningocele have been used. A total of 81 operations were performed in these patients, 69 of them at the Syringomyelia Clinic. Combinations of strategies were often chosen; the use of one strategy such as drainage did not preclude another such as transection or augmentation of the cerebrospinal fluid pathways. The overall postoperative complication rate was 12%. Problems specific to the operation type included dislodged, blocked, and infected drains (10 patients). Acute gastric dilation was seen following pedicled omental graft (one patient). At 6 years only 49% of the drains inserted still functioned. A higher than expected rate of cervical spondylotic myelopathy has been noted. Two patients developed Charcot's joints. Thirty-six patients were asked to score themselves with regard to limb function and performance of daily living activities and 30% reported improvement, particularly ion arm function. Since the use of magnetic resonance imaging has become widespread, it has become apparent that decompressive laminectomy with subarachnoid space reconstruction is effective in controlling the syrinx cavity. In complete paraplegia, spinal cord transection is an effective alternative. Pedicled omental grafting was associated with poor outcome and an increased complication rate and has been abandoned.

Activities of Daily Living↗
PubMed2000

Successful neuroendoscopic third ventriculostomy for hydrocephalus and syringomyelia associated with fourth ventricle outlet obstruction. Case report.

The authors report the use of neuroendoscopic third ventriculostomy to treat successfully both hydrocephalus and syringomyelia associated with fourth ventricle outlet obstruction. A 27-year-old woman presented with dizziness, headache, and nausea. Magnetic resonance (MR) imaging demonstrated dilation of all ventricles, downward displacement of the third ventricular floor, obliteration of the retrocerebellar cerebrospinal fluid (CSF) space, funnellike enlargement of the entrance of the central canal in the fourth ventricle, and syringomyelia involving mainly the cervical spinal cord. Cine-MR imaging indicated patency of the aqueduct and an absent CSF flow signal in the area of the cistema magna, which indicated obstruction of the outlets of the fourth ventricle. Although results of radioisotope cisternography indicated failure of CSF absorption, neuroendoscopic third ventriculostomy completely resolved all symptoms as well as the ventricular and spinal cord abnormalities evident on MR images. Neuroendoscopic third ventriculostomy is an important option for treating hydrocephalus in patients with fourth ventricle outlet obstruction.

Adult↗
PubMed2002

Hajdu-Cheney syndrome and syringomyelia. Case report.

This 7-year-old boy with Hajdu-Cheney syndrome presented with cervical syringomyelia related to rapidly progressing platybasia. Decompressive craniectomy provided temporary improvement, and his clinical status was eventually stabilized after external immobilization, according to findings at 2.5 years of follow up. In a review of the literature the authors found 57 cases of the syndrome, only three of which were associated with syringomyelia. The youth of the patient, the severe form and rapid course of the disease, and the very specific anatomical conditions related to cranial and facial deformities raised various therapeutic problems.

Child↗
PubMed2001

A craniocervical injury-induced syringomyelia caused by central canal dilation secondary to acquired tonsillar herniation. Case report.

The authors report on a 19-year-old man with an acquired tonsillar herniation caused by a craniocervical junction injury in which serial magnetic resonance (MR) images demonstrated patent and isolated segments of the central canal participating in the dilation and then formation of a cervical syrinx. The patient was involved in a motor vehicle accident; he developed tonsillar herniation as a complication of subarachnoid and epidural hemorrhage, predominantly observed around the cisterna magna and upper cervical canal. Repeated MR images obtained over an 11-month period indicated the for mation and acute enlargement of the syrinx. Ten months after the accident, the patient presented with sensory disturbance in both upper extremities and spasticity due to syringomyelia. He underwent craniocervical decompressive surgery and doraplasty, which reduced the size of syringomyelia. The authors postulate that the patent central canal may play a role in determining the location of a syrinx remote from a focus of cerebrospinal fluid obstruction.

Adult↗
PubMed2002

Syringomyelia caused by loosening of multistrand cables following C1-2 Brooks-type fusion in the rheumatoid cervical spine. Case report.

The authors report a very rare case of syringomyelia caused by loosening of multistrand cable wires following C1-2 Brooks-type fusion in a 36-year-old woman with a 13-year history of rheumatoid arthritis (RA). The syrinx vanished immediately after removal of the cables, and 2 years later no recurrence of symptoms or deterioration has occurred. The authors contend that multistrand titanium cables should not be used to fix a graft-assisted C1-2 construct in patients with RA, although this material is good for fixing rods. The phenomenon observed in this case adds to our understanding of the pathogenesis of noncommunicating syringomyelia.

Adult↗
PubMed2006

Syringomyelia associated with intradural arachnoid cysts.

OBJECT: Intradural arachnoid cysts are relatively uncommon pouches of cerebrospinal fluid (CSF) found within the subarachnoid space. The authors present a series of eight symptomatic patients in whom syrinx cavities were associated with arachnoid cysts, and they discuss treatment strategies for this entity. METHODS: The population comprised eight men whose mean age was 50 years (range 35-81 years). All patients experienced gait difficulty, and it was the chief complaint in seven; arm pain was the primary complaint in one. No patient had a history of spinal trauma, meningitis, or previous spinal surgery at the level of the syrinx cavity or arachnoid cyst. In each patient imaging revealed a syrinx cavity affecting two to 10 vertebral levels. Posterior thoracic arachnoid cysts were found in proximity to the syrinx cavity in each case. There was no evidence of cavity enhancement, Chiari malformation, tethered cord, or hydrocephalus. All patients underwent thoracic laminectomy and resection of the arachnoid cyst wall, and postoperative neurological improvement was documented in each case. The mean follow-up duration was 19 months (range 4-37 months). Follow-up magnetic resonance imaging demonstrated the disappearance of the arachnoid cyst and a significant decrease in syrinx cavity size in each patient. CONCLUSIONS: Spinal arachnoid cysts can be associated with syringomyelia, likely due to alterations in normal CSF dynamics. In symptomatic patients these cysts should be resected and the normal CSF flow restored. The results of the present series indicate that neurological improvement and reduction in syrinx cavity size can be achieved in patients with syringomyelia associated with intradural arachnoid cysts.

Adult↗
PubMed2005

Axillary vein thrombosis as a manifestation of rapidly progressive neuropathic arthropathy of the shoulder associated with syringomyelia.

Neuropathic arthropathy is a destructive joint disease associated frequently with loss of proprioception. Syringomyelia (with or without Arnold-Chiari malformation) is characterized by slow progression over many years; the joints involved most frequently are the shoulders and elbows. Neuropathic arthropathy of the hand is rare, and clinical features are vasomotor problems, digital ulcers, thickening of the fingers, and painless subluxation of the finger joints. We report an unusual case of neuropathic arthropathy of the shoulder and hands, associated with syringomyelia without Arnold-Chiari malformation. Neuropathic arthropathy of the shoulder was rapidly progressive, and axillary vein thrombosis was the first manifestation; however, neuropathic arthropathy of the hands was unrecognized for 7 years, and painless ulceration of the fingers with spontaneous amputation of the terminal phalanx and Raynaud phenomenon were the initial manifestations. To our knowledge, this is the first report of a patient with this constellation of findings.

Adult↗
PubMed2006

Application of ventriculoperitoneal shunt as a treatment for hydrocephalus in a dog with syringomyelia and Chiari I malformation.

A twenty-month-old Chihuahua male dog was presented to us suffering with ataxia. Based on the physical examination, X-ray and magnetic resonance imaging (MRI) examinations, we diagnosed the dog with hydrocephalus, Chiari I malformation and syringomyelia. Treatment consisted of internal medical treatment and the placement of a ventriculoperitoneal (VP) shunt. The ventricular dilatation was relieved and the dog improved neurologically; however, the Chiari I malformation and syringomyelia remained after surgically positioning the VP shunt.

Animals↗
PubMedDate not supplied

H-reflex recovery curve in syringomyelia.

H-reflex recovery curves were obtained from 13 patients with cervical syringomyelia to assess motor neurone excitability, and results were compared with control subjects and patients with spasticity due to stroke. The median nerve was stimulated at the elbow and the H-reflex was recorded from the flexor carpi radialis muscle. Double pulses with interstimulus intervals ranging from 75 to 900 ms were delivered. The H-reflex was unobtainable from either limb in two patients with advanced disease and loss of all sensory modalities. H-reflex recovery curves from syrinx patients showed marked facilitation with interstimulus intervals ranging from 150 to 300 ms. Facilitation was higher than in patients with spasticity due to stroke. Results are indicative of an increased motor neurone excitability in patients with cervical syringomyelia.

Adolescent↗
PubMed1999

[A clinical study on long-term outcome in patients with syringomyelia associated with Chiari malformation].

To investigate the long-term outcome in patients with syringomyelia associated with Chiari malformation (chronic tonsilar herniation), we investigated the actual factors of the patient's problems by a questionnaire for the patient. Replies to the questionnaires were obtained from 44 patients with syringomyelia who had been treated with expansive suboccipital cranioplasty with dural plasty (with plugging of the central canal in 20 patients). In 25 cases (68%) in whom the motor dysfunction of the upper extremities remained, the symptoms improved in 16 cases (43%) and were unchanged in 9 cases (24%). In 18 cases (64%) in whom the motor dysfunction of the lower extremities remained, the symptoms improved in 10 cases (36%) and were unchanged in 8 cases (29%). In 31 cases (78%) in whom the sensory disturbance remained, the symptoms improved in 19 cases (48%) and were unchanged in 12 cases (30%). The motor dysfunction of the upper extremities persisted significantly in more patients having a duration of illness over 2 years than in patients with those less than 2 years. Sensory disturbance persisted significantly in more patients with a duration of illness over 3 years than in patients with that less than 3 years. These results suggest that firstly, sensory disturbance (disturbance of the posterior horn) is most apt to remain, then motor dysfunction of the upper extremities (disturbance of the anterior horn) and followed by motor dysfunction of the lower extremities (disturbance of the pyramidal tract). We conclude that patients should be treated before having irreversible spinal cord disturbance.

Adolescent↗
PubMed1999

Treatment of syringomyelia after posttraumatic paraparesis or tetraparesis.

This retrospective study of 12 patients with syringomyelia related to spinal cord trauma with paraplegia or tetraplegia and secondary progressive neurologic deficits was conducted to evaluate various surgical treatments. Judging by the results of postoperative neuroradiologic examinations, 75% had incomplete reduction of the spinal fracture at the time of initial surgery. The secondary neurologic deterioration occurred within a delay of 146 +/- 16 months and included ascending sensory deficits in 92%, deafferentation pain in 83%, and increased motor weakness in 33%. There was a positive correlation between the severity of symptoms, incomplete reduction of spinal fracture, and the degree of arachnoid scarring in preoperative neuroradiologic examinations. Syringoperitoneal shunting was performed in 83% of patients, and laminectomy with arachnoid lysis and dural grafting were performed in 17%. Pain was improved in 75%, sensory deficits in 25%, and motor weakness in 8%. During the follow-up period of 44 +/- 25 months, 30% of patients with syringoperitoneal shunting required repeated operation for obstruction or infection, whereas the syringomyelia remained collapsed in the two patients with laminectomy with arachnoid lysis and dural grafting, but this did not require additional surgery. In conclusion, laminectomy with arachnoid lysis and dural grafting seems to be a promising alternative treatment for patients with secondary neurologic deterioration after traumatic paraplegia or tetraplegia. Syringoperitoneal shunting may be reserved for patients without severe arachnoid scarring.

Adult↗
PubMed2000

[New approaches to the evaluation of functional state of afferent central nervous system pathways in patients with syringomyelia].

Functional state of both afferent and efferent pathways of CNS was studied with the method of magnetic stimulation (MS) in patients with syringomyelia. A control group contained 35 healthy individuals. The state of the efferent pathways was estimated by the time of the central motor conduction. The state of the afferent pathways of brain stem and spinal cord was analyzed by the latency of the motor responses of the orbicular muscle of the eye to MS of the spinal cord on the level of cervical and lumbar regions. The signs of the disorders in the conduction of the excitement through pyramidal pathways as well as through the afferent pathways of the spinal cord and the brain stem were registered in patients with syringomyelia. This observation allows to increase considerably the diagnostic range of MS.

Adult↗
PubMed2001

Syringomyelia as a complication of tuberculous meningitis.

Tuberculous meningitis (TBM) is a common manifestation of extrapulmonary tuberculosis. Syringomyelia is a rare complication of TBM. We report a case of syringomyelia due to TBM. A 25 year old Thai male was admitted with a history of progressive paraparesis and loss of body sensation. He had a history of TBM in the previous year, and was treated with antituberculous drugs. Physical examination revealed a temperature of 37 degrees C. Motor power was grade 3/5 with generalized hyperreflexia. He had bilateral loss of pain, temperature and vibratory sensation below the T7 level. A magnetic resonance imaging of the spine demonstrated a long segment of syrinx from C4 to the conus medullaris region. A T12-L1 laminectomy and syringosubarachnoid shunt were done. His clinical symptoms improved after surgery.

Adult↗
PubMed2001

[Spontaneous resolution of cervical syringomyelia. A case report].

A 54-year-old man visited our hospital with complaints of severe pain in the neck and the right upper limb. His MR (magnetic resonance) imaging showed a cervical syringomyelia associated with Chiari I malformation. After one month, his complaints vanished smoothly, and the repeated MR imaging revealed shrinkage of the syrinx. The mechanisms of spontaneous resolution of syringomyelia were discussed according to some relevant literature.

Arnold-Chiari Malformation↗