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A 27-year-old White woman with syringomyelia underwent a caesarean section. The pre-operative preparation and anaesthetic management are presented, and the specific problems discussed.
Thirteen cases of syringomyelia which was confirmed by metrizamide CT myelography (MCTM) were reported. Nine cases were associated with Chiari malformation, and four were idiopathic type. Delayed CTM which were performed in several hours after the metrizamide myelography disclosed not only the existence of the syrinx but its shape and location. From the investigation of their clinical findings together with the appearance of their syrinx on CT scan, the following results were obtained. The syrinx was not always situated centrally within the cord, but had laterality at some spinal level in nine out of thirteen cases. The main laterality of the syrinx seemed to be located in the posterior horn. In the cases which had unilateral dissociated sensory disturbance, the laterality of sensory disturbance corresponded with the side of syrinx. These results suggested the possibility that the intrathecal injected contrast materials entered into the syrinx via the dorsolateral sulcus of the cord rather than via IV ventricle in some cases.
Seventy-five patients with a clinical diagnosis of syringomyelia were examined by computed tomography after intrathecal injection of metrizamide. A central cavity was demonstrated in 67 patients. Tilting the patient head down did not increase the rate of cavity opacification. This evidence favors transneural migration of metrizamide into the cavity. The spinal cord was measurably enlarged in only a minority of the patients. In some, the cavity appeared to have clefts or wall defects. These results are discussed according to the etiopathogenic theories advanced by Gardner, Aboulker, and Williams.
This sequela may be more common than is recognized. Because syringomyelia can progress to advanced stages without overt symptoms, awareness of this entity is vital for early diagnosis. Unexplained pain, numbness and ascending or descending anesthesia, followed by motor loss, point to the diagnosis. Other signs include neurotrophic arthropathy, Horner's syndrome, autonomic dysreflexia and manifestations of brainstem involvement. Computed tomography helps identify the cysts. Operative procedures to promote drainage of the fluid from the syrinx may cure or halt progression of the lesion.
We report a rare case of acute respiratory failure in a previously asymptomatic patient showing clinical signs of inferior cranial nerve palsy together with weakness and muscular atrophy of the upper limbs. Magnetic resonance imaging revealed Arnold-Chiari malformation associated with platybasia, basilar impression, syringomyelia and Klippel-Feil syndrome. Episodes of apnoea required tracheostomy and recurred upon tentative closure of the tracheostome, but remitted upon decompression of the posterior fossa. This case involved both obstructive mechanisms and dysfunction of the respiratory centre. Patients with respiratory failure not explained by pulmonary pathology should be checked for underlying neurological disease.
The paper provides the authors' experience in using neurosonography in the diagnosis of osteoneural abnormalities of the vertebral canal and craniovertebral passage in 22 patients operated on for syringomyelia and Type 1 Arnold-Chiari malformation. Comparison of computed tomographic and X-ray findings and intraoperative verification indicate that neurosonography is of high informative value and they provide evidence for its use in a diagnostic set of follow-ups of patients operated on.
The paper gives clinical findings of 5 cases of syringomyelia surgical treatment using neurotransplantation of embryonal spinal marrow nerve tissue into the intramedullary cavity. It also discusses problems of sampling, culture and transplantation of embryonal spinal marrow nerve tissues, as well as the results and prospects of this method application.
This report refers to the case of a 39-year-old woman, fourth gestation, who during her first pregnancy developed neurologic deficit of the left hand, which slowly progressed getting to affect during seven years, all the left upper extremity and the right hand. During this time, she had two abortions, and between the third and fourth pregnancies, because of the clinical suspicion, laboratory tests were done, finding by electromyography, lesion of anterior process of spinal chord at cervical level. During this fourth pregnancy, neurologic deficit increased and it was decided to interrupt pregnancy by cesarean section under epidural block at 38 weeks of gestation. According to our knowledge this syringomyelia case and pregnancy, is the first one reported in our country.
A rare coexistence of multiple intracranial neoplasms of mesodermal origin with asymptomatic syringomyelia is reported. The clinical findings were documented by NMR and CT results. The histological examination of the tumours removed surgically is included.
A 32-year-old woman experienced subacute onset of weakness in her left leg, urinary retention and difficulty in extending her right middle and third finger. She subsequently suffered episodes of myelopathy, optic neuritis and cerebellar ataxia over a period of several years. Brain MRI showed multiple areas of high signal intensity on T2-weighted images, consistent with multiple sclerosis (MS). However spinal MRI revealed no abnormal findings. In her most recent episode, at age 40 she developed paraparesis. Neurologic examination revealed down beat nystagmus on gazing to the right, horizontal jerk nystagmus gazing to the left, weakness of the right middle and third fingers and paraparesis associated with spasticity of the right leg. Sensory disturbance below C3 and diminished vibration and position sense in both legs were also observed. The patient could not stand or walk, and urinary disturbance was present. Spinal MRI revealed syrinx formation at the level of vertebral bodies C2 to C6. The syrinx within the cervical cord diminished in size after four months, but the patient was unable to walk unaided and had moderate sensory disturbance as before. This finding suggests that the prognosis of MS with syrinx formation following repeated episodes of myelopathy is not always favorable. We believe that functional recovery in MS with syringomyelia is affected by the severity of the demyelination and/or gliosis caused by MS rather than by the presence of the syrinx.
In 12 patients syringomyelia was diagnosed using MRI. The underlying cause was a hindbrain hernia in 8 patients, a posterior fossa cyst in one case, and in 3 patients the underlying cause remained unknown. Syringo-subarachnoid shunting in 8 patients failed to decrease pain and the size of the syrinx, motor lesion and gait disturbance in the majority of patients. A cysto-peritoneal shunt in the patient with a posterior fossa cyst and a syringo-peritoneal shunt resulted in a decreasing syrinx and clinical improvement.
Many neurological disorders can lead to neuropathic arthropathy. When severe, the radiographic changes are virtually pathognomonic: joint disorganization is more profound than in any other arthropathy. Neuroarthropathy of the shoulder is most frequently seen in syringomyelia and can be the presenting symptom or the symptom leading to the--previously unrecognized--diagnosis. Such a case is presented.
Optimal management of congenital and acquired spinal cord lesions, such as Chiari malformations, syringomyelia, and intramedullary spinal cord tumors, depends on an understanding of the pathogenesis and natural history of these lesions. Magnetic resonance imaging and detailed physiologic investigations are shedding new light on these difficult-to-diagnose entities, and new surgical techniques are aiding in their management.
It is tried to summarize the different opinions concerning the pathogenesis, radiobiology and techniques of irradiation in the treatment of syringomyelia. The results obtained at the Radiotherapeutic Clinic of the University of Vienna are reported. Between 1969 and 1974 a total of 25 patients with this disease were treated with doses of 500 to 1500 rd SD. In 15 patients (60%) an improvement of symptoms occurred. Long time remissions could be observed in six out of 11 patients.
In patients with syringomyelia and tuberculous spondilitis accompanied by spinal disturbances (which are in accordance with clinical data considered as models with prevalent signs of disintegration and irritation in the spinal cord) the authors studied separate parameters of the H-reflex and 2 functionally different muscles: m. gastrocnemium and m. soleus. It was possible to show certain differences in the parameters of the H-reflex between the two groups of patients and between the indices of the 2 studied motoneuron pools within each group. The report contains some ideas in relation to the possible causes of these phenomena.
The term orofacial granulomatosis was introduced for orofacial lesions which resemble Crohn's disease clinically and histologically in patients without gastrointestinal disease. The Melkersson-Rosenthal syndrome and its oligosymptomatic form Meischers granulomatous cheilitis are usually felt to be part of the same spectrum of disease. Oedema usually involves tissues of the lower half of the face. We describe a patient with histological changes consistent with those found in orofacial granulomatosis, but with isolated localized oedema of the right periorbital tissues. The patient also had syringomyelia which has not previously been described in association with orofacial granulomatosis.
The subjects were among the patients registered at the Nagoya University Hospital, Aichi Medical University Hospital and Nagoya National Hospital between 1972 and 1995. There were 21 males and 21 females in this series. Symptoms began at 10 to 55 years of age. Cranial nerves were involved in 57% of the cases. The bulbar lesion was usually unilateral and on the same side as the cervical lesion. In regard to the cardinal signs of syringomyelia, segmental dissociated sensory loss was present in 95% of cases, segmental atrophy of upper limbs in 90%, and loss of sweating in 48%. Advanced cases became chair-bound because of spastic paralysis. However, they usually had no bulbar palsy nor sphincter disturbances, and the life expectancy was close to normal with adequate nursing. In twenty-one cases undergoing operation of posterior fossa decompression, the side of greater tonsilar herniation corresponded to the dominant side of cervical lesion.
The authors describe a case of pregnancy in a patient previously affected by Arnold-Chiari malformation associated with syringomyelia; from clinical experience and a review of the literature, the possibility is deduced of carrying out the pregnancy physiologically, although by observing close precaution while performing delivery which, by our experience, should be accomplished by partus caesarius in general anaesthesia, taking great care to the evaluation of neurologic parameters.