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During July-August 1998, we encountered 4 patients who suffered from syringomyelia. The diagnosis in each case could not be reached on clinical grounds only, since the characteristic thermoanalgesia dissociation was absent in all patients. In each case the spinal MRI showed the typical intraspinal cystic lesion, which finally established the diagnosis and led to appropriate treatment.
This review reports the value of current imaging techniques in the diagnosis of syringomyelia excluding tumoral causes. The value of imaging techniques for evaluation of extension, detection of associated anomalies, and pre- and postoperative evaluation are detailed.
A case of limb hypertrophy secondary to syringomyelia is presented.
Catheterization of the Sylvian aqueduct is an elective technique for the surgical treatment of syringomyelia with Chiari's defect. It completes efficiently the decompression of the defect and makes possible exclusion of the IV th. ventricle and of the ependymal orifice of the syringomyelic cavity. The operative results appear thus more regular and more stable than those obtained with simple decompression. Intubation of the aqueduct has opportune indications in the treatment of tumors of the P.C.F. in the case of incomplete exeresis or when a secondary blockade of the spinal fluid is to be feared. Intubation of the aqueduct constitutes a useful complement for those patients treated during childhood for hydrocephalon with sylviduct stenosis and that evidence lately a poor functioning of the ventricular tube with ventricles exhibiting a reduced volume and hypereacute accidents of intra-cranial hypertension.
The need for clinical research coordinators (CRCs) has grown in recent years due to the increasingly rapid translation of scientific advances from preclinical experiments to clinical trials. CRCs perform a number of critical roles in clinical trials, such as ensuring adherence to the research protocol and careful monitoring of the study data. Although many of these duties are now standardized in a general job description, new fields of clinical research may require additional functions of the CRC that are specific to each investigation. This was the case for a pilot clinical study at the University of Florida, which investigated the feasibility and safety of human fetal spinal cord (FSC) tissue allografts in patients with progressive posttraumatic syringomyelia (PTS). The CRC for this study had several essential duties, such as arranging transportation for PTS subjects to the study center from all regions of the United States and coordinating an extensive assessment protocol that required many co-investigators. Given these challenges, successful achievement of the outcome measures required the development of a customized CRC job description that encompassed both standard roles and specific duties for this study. Accordingly, this article will illustrate the role of the CRC in this study and provide a template for similar coordinator roles.
A 22 years old girl had features of optic pathway glioma, scoliosis, Chiari type 1 malformation and cervical syringomyelia. She had no cutaneous lesions. We considered this combination to be more than coincidental and argue in favour of considering the case as a variant form of Neurofibromatosis type 1. The relevent literature in favour of our contention has been reviewed.
Three hundred and sixty four patients with Chiari malformation (CM) type I were examined: 263--with isolated CM and 101--with concomitant syringomyelia (SM). To study clinical features of the disease, neurological examination, craniography and magnetic resonance imaging of the brain and spinal cord were used. Similar predisposing factors and groups of objective neurological symptoms were found. Differences in the first subjective appearance and age at the disease onset were revealed. In patients with isolated CM, brain stem and cerebella symptoms dominated in the clinical picture, with spinal disturbances and asymptomatic cases being sometimes found. In patients with CM + SM, brain stem and spinal disturbances were more frequent and severe, comparing to those with single CM. Similar clinical features are due probably to common disease pathogenesis and the different ones are the result of the cerebrospinal fluid disturbances, causing intracranial hypertension, hydrocephaly and SM.
In order to produce syringomyelia, localized arachnoiditis was created in adult New Zealand albino rabbits and Wistar rats by the injection of kaolin into the thoracic spinal subarachnoid space and incision of the dura mater of the thoracic spinal cord. The rabbits and rats were divided into 3 groups; the control group, dural incision group (DG) and kaolin injection group (KG). Each rabbit was sacrificed at 4, 8, 12 and 16 weeks after the operation. Each rat was sacrificed at 8 and 16 weeks after the operation. Cavity formation in the cord of all rabbits was examined by ultrasound. All animals were perfused with 10% neutral beffered formalin at 150 cm H2O pressure, and histological examination was performed with Luxol fast blue (LFB) and hematoxylin and eosin (H&E) stains. Results obtained: (1) Cavity formation was noted in 6 of 16 DG of rabbit (37.5%), 5 of 16 KG of rabbit (31.2%) and 2 of 9 KG of rat (22.2%) with histological verification. With use of ultrasound, cavity was noted in 3 of 16 DG rabbits (12.5%) and 2 of 16 KG rabbits (18.8%). (2) Cavity formation was present in the cord adjacent to the marked adhesive arachnoiditis both in rabbits and in rats. (3) Cavity was noted in the ischemic area. (4) In 2 rabbits in which kaolin encircled whole surface of the spinal cord, hydromyelia was formed communicating with enlarged central canal caudad from the kaolin subarachnoid block. (5) Histological examination showed obliteration or narrowing of lumen of the small pial vessels involved in the adhesive arachnoiditis. In the cord parenchyma adjacent to the arachnoiditis, multiple spots of demyelination due secondary to ischemia demonstrated by LFB stain were noted. On the other hand, in the cord with the pia-arachnoid remained uninvolved, no demyelination was observed. (6) Localized adhesive arachnoiditis consisted of proliferation of fibrous tissue, lymphocytic infiltration and obliterating processes of small pial vessels involved in it. These data suggest that the cavitation within the cord would be induced by the ischemia, and hydromyelia would be produced by the pressure dissociation between the spinal subarachnoid space and the central canal.
A 43-year-old woman had noticed muscular weakness in her arms for four years before her admission. Muscle weakness and atrophy were prominent in the bilateral deltoid muscles, but muscular strength was almost unimpaired in the bilateral forearms and intrinsic muscles. There was no sign of sensory impairment except vibratory sensation. EMG revealed neuropathic NMU. X-P of the cervical spine showed enlargement of the spinal canal diameter, and MRI of the spinal cord revealed a large syrinx. On the basis of metrizamide CT and cranial MRI, a diagnosis of syringomyelia with Chiari malformation (type I) was made. Despite the presence of a large syrinx extending from C 1 to Th 11, the only detectable neurological sign was proximal weakness of the upper extremities simulating myopathy.
The authors review their experience of 14 children with syringomyelia and associated Chiari I malformation observed during the last 21 years. Initial symptoms were motor weakness (57%), sensory signs (50%), scoliosis (50%) and bladder dysfunction (21.5%), while signs of admission were pyramidal signs (78.5%) with motor deficit (43%), spinal deformities (64.2%), syringomyelic syndrome (36%) and sensory deficit (21.5%). Four children were investigated with myelography, three with myelography and spinal CT Scan and the last seven cases with MRI. Ten children (71.4%) underwent a decompression of the Chiari malformation with or without drainage of the cyst and shunting of an associated hydrocephalus. Syringo-peritoneal shunts were used in 3 children and laminectomy with syringo-subarachnoid shunt in 1 case. The neurological symptoms improved in 57% of the cases, 2 patients deteriorated and 4 patients were lost to follow-up. The authors discuss the clinical symptomatology, the MRI study, the controversies concerning the treatment and concluded with their attitude concerning this pathology in children.
We report on a 43-year-old female who, for 27 years, had thoracic scoliosis with curve convexity to the left side. She complained of rapid progression of the central cord syndrome in the past two years. Magnetic resonance imaging (MRI) demonstrated a syringomyelia with a direct connection between the fourth ventricle and the syrinx. The patient underwent a ventriculoperitoneal shunt and showed rapid neurologic improvement. A follow-up MRI showed dramatic shrinkage of the syrinx.
Acquired syringomyelia was diagnosed in an 11-year-old Fox Terrier with progressive paresis in the left pelvic limb. Myelography and magnetic resonance imaging confirmed an intramedullary lesion involving the left dorsolateral portion of the spinal cord at the level of L-2 and L-3 vertebrae. Clinical signs improved after surgical syringotomy. Microscopic evaluation of a biopsy specimen from the cyst wall did not establish a definitive histologic diagnosis. The cyst was decreased in size on magnetic resonance imaging 7 months later.
Caudal dysplasia sequence (CDS) comprises developmental anomalies of the caudal vertebrae, neural tube, urogenital and digestive organs, and hind limbs, the precursors of all of which are derived from the caudal eminence. Although the syndrome is well recognized, the etiology and pathogenetic mechanisms are poorly understood. We report syringomyelia in association with the CDS, which has not been described previously and recommends inclusion of this entity in the spectrum of abnormalities encountered in this syndrome.
An elderly woman developed humeral subluxation secondary to syringomyelic neuroarthopathy. Atypical features included her advanced age, the unilaterality of sensory symptoms and signs, the nearly normal motor function, and the absence of long tract signs. Magnetic resonance imaging confirmed the presence of a cervical syrinx, and electrodiagnostic studies suggested superimposed compressive brachial plexopathy. This case suggests that syringomyelia may be underdiagnosed in patients with atypical presentations.
OBJECTIVE: To evaluate the effect of Ginkgo biloba extract (GBE) on neuronal apoptosis in rabbits with kaolin-induced syringomyelia. METHODS: Twenty-four of 30 Chinese white rabbits were subjected to injection of 25% kaolin mixed with equal volume (0.6 ml) of cerebrospinal fluid drawn from the cisterna magna under ketamine anesthesia. Twelve of these 24 rabbits then received intravenous injection of 5 ml of GBE (5 ml/days for 14 days, GBE treatment group) while the other 12 were treated with the same amount of saline administered in similar manner (saline group). The 6 rabbits without kaolin treatment received a sham operation to serve as the control group. At different time points after the operation, the rabbits were killed and the spinal cord samples examined by immunohistochemistry. RESULTS: Histologically, ischemia and edema in the cervical cord of rabbits in GBE treatment group were less severe than those in saline group. TUNEL-positive and bax-positive neurons were less numerous in GBE treatment group than in saline group, and the former group showed more Bcl-2-positive neurons. The number of apoptotic neurons reached the peak level on day 7 after kaolin injection. CONCLUSION: GBE can ameliorate kaolin-induced hydrocephalus in the upper cervical cord and inhibit kaolin-induced neuron apoptosis.
A case history of a 28-year-old woman who sustained a moderately severe head injury and then developed acute bilateral arm weakness is presented. Magnetic resonance imaging studies revealed a Chiari I malformation with a large cervical syringomyelia (hydromyelia). The patient's arm weakness almost completely resolved spontaneously as did her syrinx. How this case is interpreted in light of the various theories of pathogenesis will be discussed.
132 cases of malformative syringomyelia have been studied at the C.I.E.R.M. (Interdepartmental Magnetic Resonance Center) of Bicêtre Hospital. The authors describe their technique for the exploration on the cord in case of suspected intramedullary cavitation, and emphasize the morphological and evolutive aspects of these abnormalities, whether they have been operated or not.
In Vologda Province, syringomyelia is rather prevalent (2.9 per 100,000 population). In view of the fact that the clinical symptoms are refractory to the treatment methods applied (roentgenotherapy and radioactive iodine), the combined use of apiotherapy (honey bee and flower pollen) and radon baths was attempted. It turned out to be undoubtedly effective.