1/9. Os odontoideum with cerebellar infarction: a case report.STUDY DESIGN: A case report. OBJECTIVES: To report the case of a child with os odontoideum associated with cerebellar infarction and to discuss the correlation between atlantoaxial instability with os odontoideum and vertebrobasilar artery insufficiency. SUMMARY OF BACKGROUND DATA: knowledge of the influence of atlantoaxial instability on vertebrobasilar artery insufficiency remains limited despite the publication of several reports. methods: A 5-year-old boy with ataxic gait disturbance was hospitalized in the pediatric ward. magnetic resonance imaging revealed multiple cerebellar infarctions, and cerebral angiogram showed occlusions of several branches of the basilar artery and a winding of the left vertebral artery. Stress lateral radiographs of the cervical spine showed atlantoaxial instability with os odontoideum. Posterior C1-C2 transarticular screw fixation with iliac bone graft was applied to obtain firm stability and fusion. RESULTS: There was no damage to the vertebral arteries or spinal nerves in the perioperative period. Solid union of the grafted bone and rigid stability of the atlantoaxial joint were seen on lateral flexion-extension radiographs 1 year after the operation. There has been no sign of recurrent arterial insufficiency, and the patient has been free from cerebellar dysfunction to date. CONCLUSIONS: Atlantoaxial instability may cause insufficiency of the vertebral artery as well as spinal cord injury. More attention should be paid to the possible relation between atlantoaxial instability and vertebrobasilar artery insufficiency.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
2/9. friedreich ataxia with minimal GAA expansion presenting as adult-onset spastic ataxia.Around a quarter of friedreich ataxia (FA) patients, despite being homozygous for GAA expansion within the FRDA gene, show atypical presentations. Our aim is to describe the case of three brothers with long-term follow-up suffering from late onset FA manifested with spastic ataxia. The three patients belong to a family with occipital dysplasia (OD) and Chiari I malformation previously reported by us. We have carried out serial examinations since 1977. Electrophysiological and neuroimaging studies, and molecular genetic analyses of hereditary ataxias are available in all three patients. Onset of symptoms occurred between 25 and 35 years. The clinical picture consisted of progressive spastic gait, truncal and limb ataxia, dysarthria, nystagmus, hyperreflexia with knee and ankle clonus and extensor plantar response, and mild hypopallesthesia. Ages at present vary between 50 and 59. One patient is wheelchair-bound but the other two are able to walk with support. Leaving OD aside, skeletal anomalies are not prominent. All three patients showed cardiomyopathy. MR imaging revealed atrophy of the cerebellum and spinal cord. Motor and sensory nerve conduction velocities were normal. Central conduction time of both motor and sensory pathways was delayed or unobtainable. All three patients were homozygous for the GAA expansion, the smaller expanded allele ranging between 131 and 156 repeats. Four heterozygotic carriers were detected among non-ataxic relatives including one with OD; furthermore, an asymptomatic OD patient showed normal genotype. We conclude that adult onset spastic ataxia is a distinctive FA phenotype associated with minimal GAA expansion. This phenotype represents a new cause of selective distal degeneration of central sensory axons. The present concurrence of OD and FA reflects coincidental cosegregation of two different inherited disorders.- - - - - - - - - - ranking = 0.5keywords = spinal (Clic here for more details about this article) |
3/9. diffusion-weighted MR imaging in acute spinal cord ischemia.We report diffusion-weighted (DW) MR findings for acute spinal cord ischemia in a 56-year-old patient. MR imaging obtained approximately 3 h after symptom onset demonstrated an area of hyperintensity on DW images, but no conspicuous signal abnormality on T2-weighted images in the conus medullaris. DW imaging of the spinal cord can contribute to the early detection of spinal cord vascular compromise.- - - - - - - - - - ranking = 3.5keywords = spinal (Clic here for more details about this article) |
4/9. MR imaging findings of spinal posterior column involvement in a case of miller fisher syndrome.Summary: The site of lesions causing ataxia in miller fisher syndrome (MFS) remains in dispute. A 43-year-old man manifested rapidly progressive left-sided ptosis, bilateral abducens palsy, areflexia, and severe ataxia. Initial MR imaging showed confined lesions of the cauda equina with gadolinium enhancement. A diagnosis of MFS was made, and the patient underwent immunotherapy. His ophthalmoplegia disappeared, but other symptoms remained. Five months after onset, MR imaging disclosed lesions confined to the spinal posterior column, which were considered to result from involvement of posterior nerve roots of the cauda equina and to be responsible for his remaining severe ataxia.- - - - - - - - - - ranking = 2.5keywords = spinal (Clic here for more details about this article) |
5/9. Fenestration of vertebral artery at the craniovertebral junction in down syndrome: a case report.STUDY DESIGN: Case report of a down syndrome patient with right vertebral artery fenestration and abnormalities of the craniovertebral junction. OBJECTIVES: Describe the utility of 3-dimensional computed tomography angiography for evaluating vertebral artery anomalies before surgery. SUMMARY OF BACKGROUND DATA: Previous reviews evaluating catheter angiograms identified various anomalies of vertebral artery at the craniovertebral junction. The frequency of vertebral artery anomalies is increased in patients having osseous anomalies at the craniovertebral junction. down syndrome is associated with a high incidence of bone abnormalities at the craniovertebral junction, but there have been no published reports of vertebral artery anomalies per se at the craniovertebral junction. methods: A 16-year-old woman with trisomy 21 presented with gait abnormalities and myelopathy in association with bone abnormalities at the craniovertebral junction, including hypoplastic odontoid and ossiculum terminale. Computed tomography angiography showed that right vertebral artery bifurcated after exiting the C2 transverse foramen with one branch passing through the C1 transverse foramen, whereas the other turned posteromedially and entered the spinal canal between C1 and C2. RESULTS: Occipito-C2 posterior fusion was performed with a rod and screw system. Intraoperatively, the course of the anomalous right vertebral artery was identified by Doppler angiography, and the surgical approach was modified to allow safe pedicle screw insertion while avoiding vertebral artery injury. After surgery, myelopathy resolved within 3 months. CONCLUSIONS: Before corrective surgery of craniovertebral junction anomalies in patients with down syndrome, the possibility of vertebral artery anomalies associated with abnormal craniovertebral junction anatomy should be considered. With preoperative 3-dimensional computed tomography angiography, we can precisely identify the anomalous vertebral artery and modify the surgical approach to reduce the possible risk of intraoperative vertebral artery injury in advance.- - - - - - - - - - ranking = 0.5keywords = spinal (Clic here for more details about this article) |
6/9. Reversible electroencephalographic and single photon emission computed tomography abnormalities in Hashimoto's encephalopathy.Hashimoto's encephalopathy (HE) is a well-established disease that occurs most commonly in patients with euthyroidism. We report 3 patients diagnosed with HE. Cases 1 and 2 were young females who had experienced intractable seizures and presented with cognitive impairment. Reversible slow waves on the electroencephalogram (EEG), and nearly complete recovery of cognition, were noted after steroid treatment. Case 3 was an elderly male who presented with a sudden onset of vertigo, diplopia, and recurrent gait ataxia. Reversible hypoperfusion in the upper portion of the left cerebellum, on (99m)Tc hexamethylpropyleneamine oxime (HMPAO) single photon emission computed tomography (SPECT), was noted after steroid treatment. We also review 25 cases from the literature of patients diagnosed with HE. All patients had antithyroid antibodies. A reversible inflammatory process is supported by brain magnetic resonance imaging, SPECT, EEG and cerebrospinal fluid studies. vasculitis is the most probable pathogenesis according to laboratory findings. steroids are the treatment of choice, and result in a favorable outcome when administered early in the disease course.- - - - - - - - - - ranking = 0.5keywords = spinal (Clic here for more details about this article) |
7/9. role of dynamic CT myelography in identifying the etiology of superficial siderosis.The authors report three cases of superficial siderosis with a fluid-filled spinal canal cavity communicating with the subarachnoid space. The site of communication was identified with dynamic CT myelography and the defect was surgically repaired.- - - - - - - - - - ranking = 0.5keywords = spinal (Clic here for more details about this article) |
8/9. Noncommunicating spinal extradural arachnoid cyst causing spinal cord compression in a child.Extradural arachnoid cysts in the spine are relatively uncommon causes of spinal cord compression in the pediatric population that are thought to arise from congenital defects in the dura mater. Most reports describe such cysts communicating with the intrathecal subarachnoid space through a small defect in the dura. The authors describe the case of a child who presented with spinal cord compression caused by a large spinal extradural arachnoid cyst that did not communicate with the intradural subarachnoid space. An 11-year-old girl presented with urinary urgency, progressive lower-extremity weakness, myelopathy, and severe gait ataxia. magnetic resonance imaging of the spine demonstrated a large extradural arachnoid cyst extending from T-8 to T-12. The patient underwent a thoracic laminoplasty for en bloc resection of the spinal extradural arachnoid cyst. Intraoperatively, the dura was intact and there was no evidence of communication into the intradural subarachnoid space. Postoperatively, the patient's motor strength and ambulation improved immediately, and no subsequent cerebrospinal fluid leak occurred. Noncommunicating spinal extradural arachnoid cysts are extremely rare lesions that can cause spinal cord compression in children. Because the dura remains intact, they can be removed entirely without subsequent dural repair. The authors review the literature and discuss the proposed underlying mechanisms of formation of these arachnoid cysts.- - - - - - - - - - ranking = 7.5keywords = spinal (Clic here for more details about this article) |
9/9. X-linked adrenoleukodystrophy with olivopontocerebellar atrophy.X-linked adrenoleukodystrophy (X-ALD) is a rare neurological disorder characterized by adrenal, gonadal and nervous system dysfunction. patients usually develop spinal cord degeneration with involvement of the cerebral white matter. While a spinocerebellar variant has been described, the selective involvement of cerebellar white matter is very rare. We report the case of a patient affected by X-ALD whose clinical and magnetic resonance imaging (MRI) results resembled olivopontocerebellar atrophy. He was a 29-year-old mentally retarded man, who began to complain of slowly progressive gait ataxia after an 8-year history of Addison's disease. Serial MRI revealed marked cerebellar atrophy involving the inferior cerebellar vermis and brainstem, but sparing the supratentorial white matter. The diagnosis of X-ALD was confirmed by elevated levels of very long-chain fatty acids in the serum. After 2 years follow-up, the patient developed spastic paraparesis. The patient represents an unusual clinical presentation of X-ALD, as further confirmed by the MRI results. Consequently, cerebellar symptoms should be considered as a clinical presentation of X-ALD. Early recognition of this rare disorder would be useful for genetic counselling and therapy.- - - - - - - - - - ranking = 0.5keywords = spinal (Clic here for more details about this article) |