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[Pathogenesis of syringomyelia in the light of our observations].

The author treated surgically 10 patients with syringomyelia. In 6 cases Arnold--Chiari syndrome was found, in 2 cases platybasia with basilar impression, besides that 2 patients had complete or partial occlusion of Maganide's foramen and in 4 cases a persisting central canal communicated widely with the 4th ventricle. In 3 cases these findings were associated with postinflammatory changes. In only one case the changes in the atlanto-occipital area could not have been defined clearly. The author compares his observation with those reported by others reaching the conclusion that they confirm the hydrodynanic mechanism of syringomyelia. In most cases syringomyelial is a result of congenital or congenital and acquired malformations in the medullo-bulbar area leading to distrubances in the circulation of cerebrospinal fluid.

Arnold-Chiari Malformation↗

Post-traumatic syringomyelia.

Post-traumatic syringomyelia is uncommon. It is seen predominantly after injury to the thoracic or the lumbar spine and rarely after injury to the cervical spine. In this report, three cases of post-traumatic syringomyelia are presented. The injury was limited to the thoracic spine in one case and to the cervical spine in the rest. The symptoms began after two years in two cases and after fifteen years in the third. The diagnosis was suggested by radiographic examination and was confirmed at operation in each case. Following evacuation and drainage of the cyst, significant improvement occurred in two patients.

Adolescent↗

[A case of spinal sarcoidosis complaining of chest and back pain as a first manifestation and mimicking syringomyelia on MRI].

The patient was a 24-year-old female complaining of bell-shaped chest and back pain with visual disturbance. Chest X-ray showed bilateral hilar lymphadenopathy without the presence of pleural effusion. Bronchoalveolar fluid showed lymphocytosis with an elevated CD 4/CD 8 ratio. Transbronchial lung biopsy demonstrated a non-caserous granulomatous lesion with an accumulation of epitheloid cells, suggesting lung sarcoidosis. No abnormality of electrocardiogram was detectable, and spinal tap for examination of chest and back pain demonstrated on elevated level of beta 2-microglobulin, and a normal angiotensin converting enzyme level. Spinal MRI showed a lineal lesion mimicking syringomyelia on T 2-weighted image. Steroid administration was started for the chest and back pain, since the spinal lesion was suspected due to spinal sarcoidosis. All clinical and laboratory findings, without the presence of pleural effusion or cardiac fluid, supported the diagnosis of spinal sarcoidosis causing chest and back pain. In the literature, patients with spinal sarcoidosis manifesting chest and back pain and with a MRI finding mimicking syringomyelia have been rarely reported. This case might be important in considering spinal cord sarcoidosis as a differential diagnosis of chest and back pain.

Adult↗

[Clinical and neuro-visual study of syringomyelia].

One hundred fifty one patients with syringomyelia of different duration were studied using magnetic resonance imaging (MRI). An association between syringomyilia cavitation and Arnold--Chiari malformation as well as cerebellar tonsils location was found. No definite relationship between syringomyelia course and degree of cerebellum ectopia was detected. With disease duration increasing, cross dimensions of spinal cord cavitation are getting smaller. This may be due to spontaneous cavitations collapse. The hypothesis is supported by flattened out cavitation phenomenon found by MRI on the axial spinal cord sections of 39 patients. Such deformation corresponds to spinal cord atrophy developing during long-term disease course.

Adult↗

[The clinical picture and pathogenesis of spontaneous biceps tendon ruptures in syringomyelia patients].

The report concerns a description of 23 patients with typical syringomyelia with ruptures of the distal ligaments of the bicapital muscle and the ligament of its long capita. The authors believe that the absence of bone changes, an increase of the level of neuramine acid and the titre of hyaluronidase in the blood serum testify to the fact that such phenomena may appear in conditions of a segmentaly neurodystrophy of connective tissues due to deafferentiation. In this aspect ligament ruptures are considered and evaluated for professional fitness and diagnosis of the initial or mildly expressed forms of syringomyelia.

Female↗

Secondary holocord syringomyelia with spinal hemangioblastoma: a report of two cases.

Intramedullary spinal hemangioblastoma is well known to be accompanied by syringomyelia. However, holocord secondary syringomyelia is uncommon. We present 2 cases of spinal hemangioblastoma, one in the conus medullaris and the other in midthoracic region, accompanied by holocord syrinx. In both the cases the secondary syrinx resolved following successful total tumor excision with good neurological recovery.

Adolescent↗

Atlantoaxial subluxation and syringomyelia in Down syndrome: report of one case.

Atlantoaxial instability is a relatively frequent finding in Down syndrome, but syringomyelia is a rare symptom. A four-year-old girl with Down syndrome was noted to have one year history of torticollis and progressive motor deterioration presenting with abnormal gait, ataxia or quadriparesis for the past three months. She was admitted because of acute urinary retention. Physical examination showed distended urinary bladder, hyperreflexia, bilateral ankle clonus and the existence of Babinski's signs. The x-ray films of the cervical spine showed widening between the joint space of atlas and odontoid processes. Magnetic resonance imaging revealed C1-C2 subluxation with syringomyelia. After bladder training and cervical orthosis, the symptoms improved.

Atlanto-Axial Joint↗

The nature, meanings, and dynamics of lived experiences of a person with syringomyelia: a phenomenological study.

Syringomyelia, considered a rare neurological disease, is relatively uninvestigated in the nursing literature. The aims of this qualitative phenomenological case study were to discover the nature, meanings, and dynamics of lived experiences of a 52-year-old Caucasian male with syringomyelia. Using van Manen's Method of Phenomenological inquiry (van Manen, 1990), data were collected, checked, and analyzed according to the philosophy, approach, and methodological procedures of phenomenology. Findings revealed an overarching theme of engulfment by disease. Essential themes included loss of abilities, struggles to adapt to changes, and life as a person who was disabled. Eleven sub-themes were also identified. Implications for nursing practice are discussed.

Activities of Daily Living↗

[Anomalous excretion of glycosaminoglycans in patients with syringomyelia].

Glycosaminoglycan (GAG) urine concentrations in syringomyelia patients were much higher than in healthy subjects from January to September. Maximum values of GAG excretion (twice as much as control) in affected patients were observed in spring and summer (from March to July). The results suggest that syringomyelia is accompanied by disorders in GAG connective tissue accumulation.

Adult↗

[Neurophysiological pain and sensory disorders correlates in patients with syringomyelia].

Short-latent and pain-related long-latent somatosensory evoked potentials (SEP) in response to electrical stimulation of n. medianus, n. tibialis and n. mandibularis were studied in 28 patients with syringomyelia. During stimulation in the area of pain-temperature sensitivity disturbance, reduced amplitude of early components N13, N18 and late components P3, N3, P4, N4 (100-500 ms) was detected. In patients with neuropathic pain, increased amplitude of the middle latency waves P2, N2 (40-100 ms) and low amplitude ratio P4/P2 (normal values = 1.5) were found. Patients with stimulus-evoked pain (allodynia and hyperaesthesia) had shortened N18 peak latency, decreased motor threshold and grossly attenuated and delayed P3, N3, P4 (100-350 ms) in SEP after stimulation of the hypersensitive side. In patients without pain, the amplitude of the late tibial SEP was elevated. The results obtained can be used as diagnostic criteria of syringomyelia. The character of SEP changes in patients with neuropathic pain support the hypothesis of central sensitization and abnormal pain transmission through the fast conducting of pain and temperature afferents.

Adult↗

[Headache and fainting as initial symptoms of syringomyelia associated to Arnold-Chiari and facial angiomatous nevus].

INTRODUCTION: Syringomyelia and Arnold-Chiari malformation are two dysraphism that often appear together. Clinical manifestations are diverse although some patients remain asymptomatic for years. Syncope, supposedly due to a dysfunction of medulla baroceptors and spinal cord intermediolateral horn, is uncommon, particularly as the initial symptom. CASE REPORT: 34 year old woman, with a facial angiomatous nevus, presented with two episodes of headache followed by syncope; neurological examination was apparently normal. MRI showed mild supratentorial hydrocephalus, Arnold-Chiari malformation and cervical syringomyelia, with no vascular anomalies. CONCLUSION: This case, which shares some features with Sturge-Weber syndrome and PHACE syndrome, enhances the importance of the search for anomalies in patients with paroxistical symptoms and cutaneous lesions.

Adult↗

[Coexistence of syringomyelia and extramedullary intradural meningioma].

INTRODUCTION: The coexistence of non-communicating syringomyelia with extramedullary intradural tumours in surgical series is extremely rare. CASE REPORT: We report the case of a 68-year-old female who had been suffering from predominantly proximal asymmetrical progressive paraparesis, with no involvement of the sphincters; the clinical exploration revealed a band of thermal hypaesthesia D7-D8. Magnetic resonance imaging (MRI), following the administration of gadolinium, confirmed the coexistence of an extramedullary intradural expansive lesion, which had features suggesting it could be a meningioma, and a dorsal syringomyelia. The cavity involved two medullary segments situated above the tumour and there were no associated anomalies at the junction of the medulla and upper spinal cord. Following complete resection of the tumour, which was diagnosed histologically as being a transitional meningioma with abundant psammoma bodies, the patient recovered the ability to walk independently. CONCLUSIONS: MRI with contrast is the best diagnostic means available for the detection and delimitation of the two components in this rare association, although its capacity to detect signs suggesting turbulences in the intracavity fluid, especially in small cavities, has still to be confirmed. The variations in the size and situation of the cavity with respect to the seat of the tumour, and the fact that progress is seldom monitored with MRI in the cases published to date, do not allow us to identify the pathogenic mechanism responsible for this association or to suggest the best type of shunt to prevent a possible expansion of the syringomyelic cavity.

Aged↗

Syringomyelia. A neuropathological study of 18 autopsy cases.

Eighteen autopsy cases of syringomyelia were studied neuropathologically. In six cases associated with Chiari II malformation, the central canal was patent from the fourth ventricle to the syrinx, and the syrinx was simply a dilated central canal. In four cases associated with Chiari I malformation, the syrinx was irregularly shaped and communicated with the subarachnoid space at the entry zone of the posterior nerve roots. In six cases associated with spinal cord or posterior fossa tumors, the syrinx was located adjacent to the tumor tissues, and occupied the medullary gray matter or the spinal intermediate zone and the ventral part of the posterior horn. With regard to the pathogenesis of syringomyelia, we concluded that in cases associated with Chiari II malformation, vermian protrusion and direct continuity between the fourth ventricle and the syrinx were essential. In cases associated with Chiari I malformation, in addition to tonsillar protrusion, communication between the syrinx and the subarachnoid space was thought to play an important role, and in cases associated with tumors, the circulatory disturbance due to the presence of the tumors caused the syrinx.

Aged↗

Syringomyelia as a complication of tuberculous meningitis.

Tuberculous meningitis may rarely be followed by the development of syringomyelia despite appropriate chemotherapy. In the present paper, we report a case of tuberculous meningitis in a 23-year-old Vietnamese male complicated by a rapidly progressive myelopathy due to granulomatous arachnoiditis which culminated in the development of a syrinx. The relevant literature is reviewed. The present case supports the hypothesis that vasculitic thrombosis of spinal cord vessels leading to ischemic myelomalacia is the mechanism causing postinflammatory syringomyelia.

Adult↗

[Late decompression in a patient with post-traumatic syringomyelia].

Syringomyelia is an uncommon, but disabling complication of spinal cord injuries. The authors presented a female patient with syringomyelia, in whom diagnosis was made 14 years after the initial trauma and decompression was performed three years thereafter. Decompression was carried out via the anterior approach without any complication. The result at the end of a three-year follow-up was satisfactory with improvement in her neurologic symptoms. This case indicates that decompression can be of benefit even though it is performed late after the occurrence of neural compression and emphasizes the need for a thorough examination of the symptoms before planning treatment.

Adult↗

Significant scoliosis regression following syringomyelia decompression: case report.

We present the case of a 5-year-old boy presenting with a 54-degree scoliosis secondary to a Chiari I malformation with a holocord syringomyelia extending from C1 to T10. Neurosurgical treatment involved posterior fossa craniectomy with decompression, and partial C1 laminectomy. At follow-up 7 years later, at age 12, radiographs revealed only a 4-degree scoliosis, and follow-up MRI revealed a deflated syrinx. We report this case to reveal the most significant scoliosis regression seen in our experience that may occur in younger patients after neurosurgical syringomyelia decompression for Chiari I hindbrain herniation.

Cervical Vertebrae↗

[Biopolymers in urine of patients with syringomyelia].

Urine of patients with syringomyelia was analysed by gel filtration, ion-exchange chromatography and analytical chemistry methods. Increased level of acidic glycoproteins and decreased level glycosaminoglycans levels were found. Patients with syringomyelia had smaller glycosaminoglycans components of proteoglycans than normal subjects.

Adolescent↗

[Communicating syringomyelia and syringobulbia after surgery of a spinal epidural tuberculoma].

In a 46 year old patient a syringomyelia and syringobulbia were diagnosed clinically 18 years after a transverse lesion of the cord and removal of an epidural spinal tuberculoma. Post-myelographic CT and NMR scans showed a communicating syringomyelia and syringobulbia. A flavectomy was performed and a syringopleural shunt implanted. The operation was successful by neuroradiological criteria. Clinically there was a worsening of the spastic gait during one-year follow up. Neuroradiological findings and etiology of the syrinx are discussed, and a causal relationship to the tuberculoma is proposed.

Brain Stem↗