1/126. Pediatric syringomyelia with chiari malformation: its clinical characteristics and surgical outcomes.BACKGROUND: Most reports regarding pediatric syringomyelia have focused not only on Chiari malformation, but also on spinal dysraphism. However, the clinical characteristics of syringomyelia with spinal dysraphism are quite different from those of syringomyelia due to Chiari Type I malformation. The objectives of this study were to identify clinical characteristics of pediatric syringomyelia and to determine whether surgery prevents or corrects the scoliosis associated with syringomyelia. methods: We reviewed the records of 16 pediatric patients with syringomyelia and Chiari Type I malformation. The patients' ages ranged from 3 to 15 years, with mean age of 9.8 years. None of the patients had spinal dysraphism. RESULTS: Nystagmus was observed in 2 of the 16 patients, motor weakness in 8 patients, sensory disturbance in 10 patients, and scoliosis in 13 patients. As the initial surgical procedure, foramen magnum decompression (FMD) was performed in seven patients and syringo-subarachnoid (S-S) shunting in nine patients. The motor function improved in 7 of the 8 affected patients, and the sensory disturbance improved in 9 of the 10 affected patients. The magnetic resonance images obtained after the surgery revealed marked decrease of the syrinx size in all patients. Of the 13 patients with scoliosis, 5 showed improvement, 5 stabilization, and 3 deterioration. CONCLUSIONS: Compared with adolescent and adult syringomyelia, pediatric syringomyelia shows a much lower incidence of sensory disturbance and pain, but quite a high incidence of scoliosis. Surgery is effective in improving or stabilizing scoliosis in these patients.- - - - - - - - - - ranking = 1keywords = pain (Clic here for more details about this article) |
2/126. Headache in Chiari malformation: a distinct clinical entity?A relatively common group of developmental anomalies of the posterior fossa and its contents make up the Chiari malformations. The most common of these anomalies are the Chiari malformations type I and type II. Many patients with these defects are asymptomatic, but many of them suffer from headaches. Although the prevalence of common primary headache disorders in patients with Chiari malformations is similar to that of the general population, a particular type of occipital headaches occurs more frequently in patients with a Chiari malformation. In the two patients described in the illustrative case presentations, the major complaint was headache. Both had Chiari malformation type I diagnosed. Chiari malformation should be considered in the differential diagnosis of patients who complain of exertional headache. Exertional headaches or worsening of pain during Valsalva's maneuver should prompt clinical suspicion of an underlying intracranial lesion and warrants further evaluation by appropriate neuroimaging studies.- - - - - - - - - - ranking = 330.82763799251keywords = headache, pain (Clic here for more details about this article) |
3/126. Arnold Chiari Type I malformation presenting as a trigeminal neuralgia: case report.OBJECTIVE AND IMPORTANCE: Arnold Chiari Type I malformation usually presents as headache, arm numbness, dysesthesia, upper weakness, or gait difficulty. We report a case of Chiari malformation presenting as a left trigeminal neuralgia. CLINICAL PRESENTATION: A patient with a history of 29 years of trigeminal neuralgia was admitted. He was treated with three thermocoagulations. Microvascular decompression was planified. magnetic resonance imaging was performed, and it demonstrated an Arnold Chiari malformation. After surgery, the patient was asymptomatic. INTERVENTION: Posterior fossa decompression by enlarging the foramen magnum and aspiration of the cerebellar tonsils was performed. CONCLUSION: The trigeminal neuralgia could be attributable to a compression of the trigeminal nucleus. The compression of the nucleus could explain both the pain and the regression after surgery. This is the second reported case of pure trigeminal neuralgia in Arnold Chiari malformation.- - - - - - - - - - ranking = 56.566275255109keywords = headache, pain, upper (Clic here for more details about this article) |
4/126. Pseudo Chiari type I malformation secondary to cerebrospinal fluid leakage.cerebrospinal fluid (CSF) leakage may occur spontaneously, iatrogenically or from spinal trauma. Postural headache is the cardinal symptom; dizziness, diminished hearing, nausea and vomiting are additional symptoms. In neurological examinations cranial nerve palsies may be found. Due to low CSF pressure neuroimaging studies may reveal dural enhancement and vertical displacement of the brain. We describe a patient with the history of an uncomplicated lumbar discectomy at the level L4-5 and the typical clinical symptoms of intracranial hypotension. MRI of the craniocervical junction showed typical features of a Chiari type-I malformation. After neurosurgical ligation of a CSF leak at L4-5 caused by lumbar disc surgery, the patient was free of orthostatic headache. A repeated MRI showed a striking reduction of the previous downward displacement of the cerebellar tonsils and pons.- - - - - - - - - - ranking = 109.9425459975keywords = headache (Clic here for more details about this article) |
5/126. shoulder enlargement as the presenting sign in syringomyelia. Report of two cases and review of the literature.Two adult patients had painful enlargement of the shoulder and complete destruction of the humeral head. Angiograms of the shoulder showed highly vascular lesions in both patients. Both patients had syringomyelia, for which the swollen shoulder was the first manifestation.- - - - - - - - - - ranking = 1keywords = pain (Clic here for more details about this article) |
6/126. Paroxysmal rage as a presenting symptom of the Chiari I malformation. Report of two cases.Signs of meningeal irritation including occipital and cervical pain are common in symptomatic children with the Chiari I malformation. The author reports on two children with Chiari I malformations who presented with a previously undescribed symptom presumably caused by intermittent meningeal irritation: paroxysmal rages. In both cases the rages stopped after decompressive surgery. Evaluation including magnetic resonance imaging should be considered in children with new onset of paroxysmal behavioral disorders.- - - - - - - - - - ranking = 1keywords = pain (Clic here for more details about this article) |
7/126. Brainstem dysfunction in chiari malformation presenting as profound hypoglycemia: presentation of four cases, review of the literature, and conjecture as to mechanism.OBJECTIVE: We report four patients whose cases resulted in our observation that profound hypoglycemia resulting from intermittent hyperinsulinism plays a significant role in patients with brainstem dysfunction from Chiari I or II malformations who have intermittent autonomic dysfunction ("blue spells"). methods: The records of four children with severe brainstem dysfunction associated with hindbrain herniation (Chiari I or II malformation) were reviewed retrospectively. Each patient had severe lower cranial nerve dysfunction that required tracheotomy and feeding tube placement. After we found that profound hypoglycemia had occurred during a spell of autonomic dysfunction in one patient, the charts of the other three patients were reviewed for evidence of hypoglycemia. Now, whenever one of them has evidence of autonomic dysfunction, prospective studies of glucose and insulin levels are performed. Three of the patients had Chiari II malformation in association with myelomeningocele, and one patient had a Chiari I malformation resulting from Pfeiffer's syndrome. RESULTS: hypoglycemia occurred in these patients episodically, and usually when their shunts were functioning. The hypoglycemia was associated with hyperinsulinemia in each patient. The brainstem structures of these children (presumably the dorsal motor nuclei of the vagus) were extremely sensitive to changes in local or regional intracranial pressure. These changes were triggered by intermittent shunt failure, agitation from pain, abdominal distention from constipation, and retention of CO2. In patients with Chiari malformations, even mild increases in intracranial pressure lead to brainstem dysfunction. One possible explanation is that pressure on the deformed Xth cranial nerve nuclei may lead to insulin release and life-threatening hypoglycemia. Continuous-drip feeds are necessary to prevent this complication. CONCLUSION: patients with severe intermittent brainstem dysfunction after decompression of Chiari I or Chiari II malformations should have laboratory studies of glucose levels performed at the time of the episodes to rule out hypoglycemia.- - - - - - - - - - ranking = 1keywords = pain (Clic here for more details about this article) |
8/126. arnold-chiari malformation with syringomyelia in an elderly woman.PRESENTATION: A 76-year-old woman, complaining of leg pain and unsteady gait for 3 years, presented with a spastic paraparetic gait, severe spasticity and touch, thermal and pain sensory loss limited to arms, lower thorax and upper abdomen. brain and spinal cord magnetic resonance imaging showed a large loculated syrinx. Cerebellar tonsillar herniation into the foramen magnum was also seen (arnold-chiari malformation, type I). OUTCOME: The patient had successful cervico-spinal surgical decompression which resulted in marked reduction in hypertonia and weakness, normal gait and normal joint movement at 6 months. CONCLUSION: This unusual, late clinical presentation of a congenital disease underlines the importance of a comprehensive diagnostic work-up in the elderly patients with complex neurological signs.- - - - - - - - - - ranking = 2.5950022563574keywords = pain, upper (Clic here for more details about this article) |
9/126. Chiari malformation and syringomyelia in monozygotic twins: birth injury as a possible cause of syringomyelia--case report.A 26-year-old female, the elder of monozygotic twins, presented with slow progressive numbness and pain in her left arm. Magnetic resonance (MR) imaging showed syringomyelia with Chiari malformation. The patient's birth had been difficult with prolonged delivery time, breech delivery, and neonatal asphyxia. MR imaging of the patient's twin sister showed mild tonsillar ectopia, but absence of syringomyelia. This younger sister was born without problems. The patient underwent syringosubarachnoid shunt at the C5-6 level. The syrinx was collapsed promptly, and her symptoms disappeared. This case of syringomyelia with Chiari malformation in one of twins suggests that birth injury is likely to be a cause of the pathogenesis of syringomyelia.- - - - - - - - - - ranking = 1keywords = pain (Clic here for more details about this article) |
10/126. Isolated Horner's syndrome and syringomyelia.Although syringomyelia has been associated with Horner's syndrome, it is typically associated with other neurological findings such as upper limb weakness or numbness. A patient is described who had an isolated Horner's syndrome as the only manifestation of syringomyelia. A 76 year old woman was discovered to have right upper lid ptosis and right pupillary miosis. Neurological examination was unremarkable, and pharmacological testing was consistent with localisation of the lesion to a first or second order sympathetic neuron. neuroimaging disclosed a Chiari I malformation with a syrinx extending to the C2 to C4 level. An isolated Horner's syndrome may be the presenting manifestation of syringomyelia.- - - - - - - - - - ranking = 1.1900045127149keywords = upper (Clic here for more details about this article) |
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