1/13. Orofacial pain as the sole manifestation of syringobulbia-syringomyelia associated with arnold-chiari malformation.This is a case report of a male patient who presented with orofacial pain for a year as the only manifestation of syringobulbia-syringomyelia associated with arnold-chiari malformation. This article places emphasis on the clinical presentation and possible differential diagnoses. The pain was continuous and affected the left side of the face. It was exacerbated by coughing and physical effort, possibly as a consequence of an increase in intracranial pressure. Paroxysmal pain crises developed over this background of continuous pain, compatible with neurogenic trigeminal pain of the left second branch, together with pain episodes similar to cluster headache on the same side. The symptoms were resolved following neurosurgical management with amplification of the foramen magnum.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/13. Anaesthetic implications of Nager syndrome.Nager acrofacial dysostosis is an oromandibular hypogenesis syndrome with associated limb abnormalities. Although it shares some phenotypic features with Treacher-Collins syndrome, it is recognized as a separate disorder. The physical features of Nager syndrome include down slanted palpebral fissures, malar hypoplasia, a high nasal bridge, atretic external auditory canals, cleft palate and micrognathia. Preaxial limb malformations include absent or hypoplastic thumbs, hypoplasia of the radius and shortened humeral bones. Of primary concern to the anaesthetist are the midface and mandibular manifestations which may complicate perioperative airway management. These problems may also manifest in the postoperative period with airway obstruction. Associated defects have included vertebral malformations with reports of cervical spine involvement, congenital cardiac defects and upper limb defects affecting the preaxial or radial side. We describe a 7-year-old boy with Nager syndrome who required anaesthetic care during placement of a syringopleural shunt for drainage of a spinal cord syrinx. The perioperative implications of this disorder are reviewed.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/13. syringomyelia presenting as paroxysmal arm posturing resembling seizures.A 9-year-old female presented with daily episodes of medically refractory paroxysmal bilateral arm posturing, which had long been thought to be epileptic seizures. She also had other types of episodes, including daily staring spells and infrequent generalized tonic-clonic convulsions. neurologic examination was normal except for delayed cognitive development. The results of previous electroencephalograms (EEG) were normal, and magnetic resonance imaging of the head revealed a Chiari I malformation. Video EEG monitoring revealed no EEG changes during the attacks, and magnetic resonance imaging of the spine revealed a large cervical syrinx associated with the Chiari malformation. The episodes of paroxysmal bilateral dystonic arm posturing resolved after surgical intervention for the syrinx. This report illustrates that cervical cord disease is an unusual although potentially treatable condition to be considered in the differential diagnosis of paroxysmal episodes with dystonic movements of the arms, even in the absence of other physical findings of myelopathy.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/13. Misinterpretation of regional sensory loss in an injured worker: a case report.Regional patterns of motor or sensory loss are considered indicators of a behavioral component to the complaints of an injured worker. This assumption may lead to a discounting of signs and symptoms with premature return to work and discharge from care. We present the case of a 25-year-old airline baggage handler with an 8-month history of unresolved neck and shoulder complaints who had been returned to work after a lack of objective findings on physical examination. On physiatric evaluation, his unusual pattern of insensitivity to pinprick led to prompt magnetic resonance imaging that revealed a focus of increased intramedullary signal at C6 consistent with a syrinx. This case report shows the importance of a detailed neuromuscular examination coupled with appropriate diagnostic imaging in the assessment of individuals with regional sensory or motor loss so as not to miss more serious spinal cord pathology.- - - - - - - - - - ranking = 6.3890306122449keywords = physical examination, physical (Clic here for more details about this article) |
5/13. arnold-chiari malformation with syrinx presenting as carpal tunnel syndrome: a case report.A 26-year-old administrative assistant presented with 3 years of left-hand dysesthesia involving primarily the first 3 digits. Her symptoms increased at night and with keyboard use. Through 12 visits to primary and specialty care physicians over 3 years, she experienced minimal improvement with splints and moderate improvement with gabapentin. On presentation, careful questioning revealed an abrupt onset of symptoms 3 years previously, related to a 2-week episode of gastritis associated with recurrent emesis. Examination revealed a negative Tinel sign over the median nerve at the wrist, decreased left biceps reflex, positive Spurling test, and decreased sensation over the palmar and dorsal surfaces of the left hand in the C5-6 distribution. The atypical onset of symptoms, poor response to therapy, and physical findings suggested the possibility of a radicular or central neurologic etiology for the patient's hand numbness. magnetic resonance imaging demonstrated a Chiari I malformation with a syrinx extending from C2 to T10, with the greatest diameter at C4. Neurosurgical decompression led to a decrease in symptoms. A meticulous history and physical examination should be performed on patients with presumed carpal tunnel syndrome with an atypical onset of symptoms or response to therapy.- - - - - - - - - - ranking = 7.3890306122449keywords = physical examination, physical (Clic here for more details about this article) |
6/13. foramen magnum decompression for the treatment of Arnold Chiari malformation type I with associated syringomyelia in an elderly patient.STUDY DESIGN: Report of a rare case of an elderly patient with late onset of Arnold Chiari malformation type I with associated syringomyelia that was successfully treated with foramen magnum decompression. OBJECTIVE: To report this rare case along with a literature review. SETTING: Gifu, japan. methods: A 69-year-old woman with a 4-year history of dull pain in her right arm was referred to the clinic. After physical and radiographical examinations, she was diagnosed with Arnold Chiari malformation type I with associated syringomyelia. A foramen magnum decompression by the removal of the outer layer of the dura mater was performed. RESULTS: At 2 years postoperatively, MRI revealed a decrease in the size of the syringomyelia. Her symptoms had also remarkably improved. CONCLUSIONS: A rare case of symptomatic Arnold Chiari malformation type I with associated syringomyelia in an elderly woman was successfully treated with foramen magnum decompression by the removal of the outer layer of the dura mater.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
7/13. Familial Chiari type I malformation with syringomyelia in two siblings: case report and review of the literature.OBJECTIVE: Familial cases of Chiari malformation with syringomyelia are rare. The majority of the reported series and case reports detail sporadic cases. The authors report two siblings who presented with Chiari type I malformation and syringomyelia (CMI S). CLINICAL PRESENTATION: We report two sisters who each presented with scoliosis on routine school physicals. Their clinical examination was unremarkable; however, imaging studies demonstrated a Chiari malformation with syringomyelia. Both underwent cervicomedullary decompression, and follow-up imaging studies revealed resolution of the syringomyelia. CONCLUSION: A review of the literature reveals fewer than ten previous reports of familial CMI S in the past 30 years. Although rare, the existence of familial cases of CMI S suggests a genetic component to the pathogenesis of this condition in at least a proportion of patients. Neurosurgeons should be aware of the familial aggregation of CMI S.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
8/13. Electrophysiologic findings in post-traumatic syringomyelia: implications for clinical management.Traumatic spinal cord injured (SCI) patients may develop pain, new weakness and/or sensory loss due to an enlarging fluid-filled cyst in the spinal cord. The clinical history and physical exam are nonspecific and insensitive, particularly for diagnosing and monitoring recurrent or progressive post-traumatic syringomyelia (PTS). We compare the sensitivity and specificity of three electrodiagnostic tests, median and ulnar F waves, electromagnetic motor evoked potentials (MEP), and needle electromyography, with respect to syrinx by imaging (MRI or CT scan) and neurologic progression on serial clinical exams. Central motor conduction time (CMCT) calculated from scalp and spine MEP was the most sensitive diagnostic test. F waves were less sensitive and less specific than the CMCT, and did not provide evidence of syrinxes in the mid or upper cervical cord. Positive sharp wave and fibrillation potentials were the least sensitive and least specific. The CMCT is a useful adjunct to imaging studies for diagnosing and monitoring PTS.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
9/13. Posttraumatic syringomyelia associated with heavy weightlifting exercises: case report.Posttraumatic syringomyelia is a well-recognized late sequel to spinal trauma occurring in 1% to 3.2% of spinal cord injured patients. Its clinical presentation is usually marked by pain, ascending sensory loss, increased muscle weakness, and depressed deep tendon reflexes. The case of a 25-year-old man with C8 complete quadriplegia, who developed a syrinx five years after his initial injury, is presented. This patient kept a log of his daily physical workout which consisted of lifting weights of 50 to 60 pounds with his neck extensors and biceps. The diagnosis was made clinically and confirmed by magnetic resonance imaging. Repeated valsalva maneuvers from daily heavy weightlifting exercises most likely predisposed this patient to the development and extension of his syringomyelia. Dramatic improvement followed surgical placement of a subarachnoid shunt.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
10/13. exophthalmos and basilar impression. A contribution to differential diagnosis of endocrine orbitopathy.We report on a male patient with exophthalmos of unclear etiology, basilar impression, syringohydromyelia and type II arnold-chiari malformation. Two diseases involving the orbital region were to be considered in differential diagnostic terms: endocrine orbitopathy and osseous orbit dysplasia. The typical physical appearance associated with basilar impression as well as suppurative keratitis in Lagophthalmos was striking. Tetraspasticity with pareses, bulbar symptoms, proximally pronounced muscular atrophy as well as a left hemihypesthesia was shown neurologically. Although the orbit CT was normal, sonography revealed thickened ocular muscles. There was euthyroidism in diffuse goiter with negative thyroid autoantibody findings. Because of lack of definitive detection of muscular swelling, tumor, or vascular anomaly in the various images, orbital dysplasia in the context of a malformation syndrome affecting several organs is suggested as the cause of the exophthalmos.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
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