1/36. Diabetic neuropathic pain in a leg amputated 44 years previously.The mechanism of neuropathic pain in the diabetic limb is far from clear. phantom limb pain likewise is of obscure aetiology. The development of typical pain in an absent leg in a patient with diabetes many years after the amputation stimulates thought as to the mechanism, not only of neuropathic pain, but also of phantom limb pain. A 58-year-old man was diagnosed with type 2 diabetes 44 years after having undergone left below knee amputation for congenital AV malformation, at the age of 13. Eight months before the diagnosis of diabetes he began to complain of pain in the leg on the amputated side-pain very similar to that described in typical diabetic neuropathy. This was followed by similar pain in the right leg. MR scan of the spine revealed a small syringohydromyelia of the thoracic cord in addition to a prolapse of disc at L(5)/S(1) level on the left side, which was first noted 5 years previously. There were no other features of S(1) compression. The typical neuropathic character of the pain involving both the amputated and the intact limbs that developed with the diagnosis of type 2 diabetes suggest that the neuropathic pain may originate from centres higher than peripheral nerves.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/36. Cutaneous and mixed nerve silent periods in syringomyelia.OBJECTIVES AND methods: We studied cutaneous and mixed nerve silent periods (CSP, MNSP) in 4 patients with cervical syringomyelia documented by magnetic resonance imaging who on clinical examination presented with unilateral hypalgesia and hypothermesthesia. In addition, we recorded upper and lower extremity somatosensory and motor evoked potentials (SEP, MEP), and cortical silent periods. RESULTS: In all patients, CSP and the later portion of MNSP were absent or shortened on their affected side, while both were normal on their unaffected side. In all patients, SEP latencies were normal following both median and tibial nerve stimulation. In two patients, the amplitude N13 (median nerve SEP), and in one patient each the amplitudes N20 (median nerve SEP) and P37 (tibial nerve SEP) were reduced. Central motor conduction time was prolonged to abductor digiti minimi muscle in one patient on the affected side, but was normal to tibialis anterior muscle in all patients. Cortical silent periods where present bilaterally in spite of unilateral complete absence of CSP and MNSP in two subjects tested. Loss of CSP and MNSP were a sensitive parameter of spinal cord dysfunction in syringomyelia. The cervical median nerve SEP response N13 reflected gray matter involvement, while corticospinal tract dysfunction was less frequently observed. CONCLUSION: Our data suggest that CSP and later portion of MNSP are generated at the spinal level by the same small myelinated A-delta fibers, and that their central network is distinct from large diameter fiber afferents and efferents.- - - - - - - - - - ranking = 10keywords = nerve (Clic here for more details about this article) |
3/36. The role of acute decompression and restoration of spinal alignment in the prevention of post-traumatic syringomyelia: case report and review of recent literature.STUDY DESIGN: Case report. INTRODUCTION: Acute post-traumatic syringomyelia formation after spinal cord injury has been considered a rare complication. At this writing, most recent reports have surfaced in neurosurgical journals. As an entity, post-traumatic syringomyelia has not been widely appreciated. It has been confused with conditions such as Hansen's disease or ulnar nerve compression at the cubital tunnel. One study also demonstrated that the occurrence of syrinx is significantly correlated with spinal stenosis after treatment, and that an inadequate reduction of the spine may lead to the formation of syrinx. This reported case describes a patient in whom post-traumatic syringomyelia began to develop 3 weeks after injury, which improved neurologically after adequate decompression. SUMMARY OF BACKGROUND DATA: A 30-year-old man sustained a 20-foot fall at work. He presented with a complete spinal cord injury below T4 secondary to a T4 fracture dislocation. The patient underwent open reduction and internal fixation of T1-T8. After 3 weeks, the patient was noted to have ascending weakness in his bilateral upper extremities and some clawing of both hands. methods: A computed tomography myelogram demonstrated inability of contrast to pass through the T4-T5 region from a lumbar puncture. An incomplete reduction was noted. The canal showed significant stenosis. A magnetic resonance image of the patient's C-spine showed increased signal in the substance of the cord extending into the C1-C2 area. The patient returned to the operating room for T3-T5 decompressive laminectomy and posterolateral decompression including the pedicles, disc, and posterior aspect of the body. Intraoperative ultrasound monitoring showed a good flow of cerebrospinal fluid past the injured segment. RESULTS: On postoperative day 1, the clawing posture of the patient's hands was significantly diminished, and the patient noted an immediate improvement in his hand and arm strength. Over the next few days, the patient's strength in the bilateral upper extremities increased to motor Grade 4/5 on manual testing. A magnetic resonance image 4 weeks after decompression showed significant improvement in the cord diameter and signal. CONCLUSIONS: Post-traumatic syringomyelia has not been reported at so early a stage after injury. This disorder is an important clinical entity that must be recognized to prevent potentially fatal or devastating complications. As evidenced by the reported patient and the literature, if this disorder is discovered and treated early, permanent deficit can be avoided. The prevention of post-traumatic syringomyelia requires anatomic realignment and stabilization of the spine without stenosis, even in the case of complete injuries, to maintain the proper dynamics of cerebrospinal fluid flow.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
4/36. Attenuation of somatosensory evoked potentials during positioning in a patient undergoing suboccipital craniectomy for Chiari I malformation with syringomyelia.Intraoperative electrophysiologic monitoring can diminish the risk of neurologic injury by enabling the detection of injury at a time when it can be reversed or minimized. This report describes a 14-year-old girl with a Chiari's malformation type I and syringomyelia who underwent a suboccipital decompression and dural patch grafting with concurrent somatosensory evoked potentials. When the patient was turned into the prone position and the neck was flexed, the left-sided somatosensory evoked potential deteriorated. After the patient's neck was repositioned, the left median nerve potential improved but did not return to baseline. Postoperatively, the patient had decreased proprioception of her left arm, which completely resolved at 2-week follow-up. This single case report does not establish the need for routine somatosensory evoked potential monitoring. Nevertheless, deterioration of the potential in this case led directly to a change in the surgical positioning, which may have significantly reduced the chances of a permanent neurologic injury.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
5/36. syringomyelia with syringobulbia presenting only with paralysis of 9th and 10th cranial nerves.We report a case of syringomyelia with syringobulbia, in a patient who presented only with involvement of the 9th and 10th cranial nerves.- - - - - - - - - - ranking = 5keywords = nerve (Clic here for more details about this article) |
6/36. Thoracic diastematomyelia with concurrent intradural epidermoid spinal cord tumor and cervical syrinx in an adult. Case report.Diastematomyelia is a rare entity in which some portion of the spinal cord is split into two by a midline septum. Most cases occur in childhood, but some develop in adulthood. A variety of concurrent spinal anomalies may be found in patients with diastematomyelia. The authors describe a 38-year-old right-handed woman who presented with a 7-month history of lower-extremity pain and weakness on the right side. She denied recent trauma or illness. Sensorimotor deficits, hyperreflexia, and a positive Babinski reflex in the right lower extremity were demonstrated on examination. neuroimaging revealed diastematomyelia extending from T-1 to T-3, an expanded right hemicord from T-2 to T-4, and a C6-7 syrinx. The patient underwent T1-3 total laminectomies, resection of the septum, untethering of the cord, and excision of the hemicord lesion. The hemicord mass was determined to be an intramedullary epidermoid cyst; on microscopic evaluation the diastematomyelia cleft was shown to contain fibroadipose connective tissue with nerve twigs and ganglion cells. Postoperatively, the right lower-extremity pain, weakness, and sensory deficits improved. Diastematomyelia can present after a long, relatively asymptomatic period and should be kept in the differential diagnosis for radiculopathy, myelopathy, tethered cord syndrome, or cauda equina syndrome. Numerous spinal lesions can be found in conjunction with diastematomyelia. To the authors' knowledge, this is the first case in which a thoracic epidermoid cyst and cervical syrinx occurred concurrently with an upper thoracic diastematomyelia. Thorough neuraxis radiographic evaluation and surgical treatment are usually indicated.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
7/36. Maternal Arnold-Chiari type I malformation and syringomyelia: a labor management dilemma.Arnold-Chiari type I malformations consists of elongation of the cerebellar tonsils with their displacement below the foramen magnum. syringomyelia is an associated cyst that accumulates cerebrospinal fluid in the cord that can impinge on local nerve fibers. pregnant women with either of these disorders are of special concern due to the potential risk of brain stem herniation and or spinal column compression from physiological changes that occur during labor. We present two cases. The first case is a patient with syringomyelia who was admitted in labor with worsening peripheral neurological symptoms. Epidural anesthesia was placed and she underwent an uncomplicated cesarean delivery with resolution of her symptoms postpartum. The second case is a patient with an Arnold-Chiari type I malformation and syringomyelia who presented in labor. The patient had an epidural placed and was allowed to progress to complete dilation and effacement at 2 station. She underwent a successful operative vaginal delivery without voluntary maternal expulsive efforts. Both patients had uncomplicated postpartum courses. Although these are rare disorders with significant potential morbidity, labor can be managed by either mode of delivery with careful patient selection. We caution that this review has insufficient numbers of patients to address the safety and efficacy of either delivery mode but rather focuses on alternatives for delivery. This report is the first to document a case of a patient with an arnold-chiari malformation and syringomyelia successfully managed in labor with a vaginal delivery.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
8/36. A novel nonsense mutation in the ABC1 gene causes a severe syringomyelia-like phenotype of tangier disease.tangier disease is a rare autosomal recessive disorder caused by mutations in the recently identified ATP-binding cassette transporter 1 gene (ABC1). A typical clinical manifestation of tangier disease is peripheral neuropathy. Former studies differentiated between two manifestations: the more frequent mono- or polyneuropathic form and a syringomyelia-like type. It is unknown whether specific mutations in the ABC1 gene or a particular genetic background are responsible for either of these forms. A family is presented comprising a case with a severe syringomyelia-like phenotype of tangier disease and absence of cardiovascular disease. Sequencing analysis of the ABC1 gene was performed. A new homozygous C-->T transition in exon 18 was found in the index patient. This mutation results in a stop codon at position 909 (R909X) leading to premature termination of translation. Her clinically asymptomatic daughters, her sister and one of her nieces were heterozygous. sural nerve biopsies were studied in the index patient at the age of 45 and 54 years; both revealed a severe neuropathy, characterized by a subtotal and finally complete loss of nerve fibres. The entire loss of schwann cells resulted in an extraordinary form of endoneurial sclerosis. Only rare capillaries, lipid-laden macrophages and fibroblasts had survived in the endoneurium. This case appears to be unique in respect to the underlying novel mutation in the ABC1 gene and its association with complete endoneurial sclerosis of all fascicles in the sural nerve and absence of cardiovascular disease.- - - - - - - - - - ranking = 3keywords = nerve (Clic here for more details about this article) |
9/36. Preoperative radiological and electrophysiological evaluation in 100 adolescent idiopathic scoliosis patients.This is a prospective study of spinal magnetic resonance imaging (MRI), electrophysiological recordings, and neurological examinations of 100 patients admitted for surgery for adolescent idiopathic scoliosis (AIS), which was conducted to assess the prevalence of structural and functional abnormalities within the spinal cord in patients with clinically normal neurologic condition. In all patients the clinical diagnosis and intact neurological condition was ascertained by a spinal orthopedic surgeon. Full-length spinal axis MRI studies (T1/T2 sequences) and somato-sensory evoked potentials of the tibial nerves (tSSEPs) were preoperatively assessed by independent evaluators blinded to the patients' medical histories. Structural spinal cord abnormalities were found in three of 100 AIS patients on MR imaging. In one patient a Chiari malformation type 1 with an accompanying syringomyelia was diagnosed, which required a suboccipital decompression. In the other two patients small thoracic syringomyelias were diagnosed. Abnormalities of spinal cord function were detected in 68% of the 100 patients: tSSEP latencies corrected for body height were increased in 56% of the patients; pathological differences between tSSEPs on the left and right sides were present in 17% (12% in combination with a prolongation of the latency). The findings of this study indicate that MRI and electrophysiological examinations are essential to assess spinal cord abnormalities that are clinically not detectable in AIS patients. Even in patients with intact neurologic condition and clinically typical right-curved thoracic scoliosis, the possibility of intraspinal pathologies should be ruled out by MRI. It is especially important to detect structural pathologies like syringomyelia and Chiari malformation before proceeding with scoliosis surgery, as these conditions are associated with a higher neurological risk during scoliosis surgery. The electrophysiological recordings made in the present study, with the high number of pathological tSSEPs, are indicative of functional abnormalities with a subclinical involvement of the recorded neuronal pathways. The relevance of the latter findings is not yet clear, but pre-operative tSSEP examinations offer the possibility of assessing alterations in spinal cord function that are undetectable by clinical examination.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
10/36. Hiccups attributable to syringobulbia and/or syringomyelia associated with a Chiari I malformation: case report.OBJECTIVE AND IMPORTANCE: Approximately 20 to 50% of patients with syringomyelia associated with Chiari malformations exhibit cranial nerve or cerebellar symptoms. However, hiccups represent a rare clinical manifestation of this disorder. We report a case of intractable hiccups resulting from syringobulbia associated with a Chiari I malformation, which was successfully treated with foramen magnum decompression. CLINICAL PRESENTATION: We report the case of a patient who presented with syringomyelia and syringobulbia associated with a Chiari I malformation, manifested as intractable hiccups and neurological deficits. magnetic resonance imaging scans demonstrated syringobulbia in the dorsal medullary region and a large cervical syrinx from C2 to C6-C7, associated with a Chiari I malformation. INTERVENTION: foramen magnum decompression and a C1 laminectomy were performed. One month later, the intractable hiccups disappeared and the neurological symptoms demonstrated improvement. CONCLUSION: Postoperative magnetic resonance imaging scans demonstrated enlargement of the subarachnoid space in the posterior fossa and disappearance of the syringobulbia. There has been no recurrence of intractable hiccups and syringobulbia in 6 months after surgery. magnetic resonance imaging of the brainstem is an important diagnostic procedure for intractable hiccups, because syringobulbia associated with a Chiari malformation represents a surgically treatable disorder, although the incidence is low.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
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