Vitamin B12 deficiency induced myelopathy: MRI findings

Transcription

1 Vitamin B12 deficiency induced myelopathy: MRI findings Poster No.: C-3386 Congress: ECR 2010 Type: Topic: Authors: Keywords: DOI: Educational Exhibit Neuro J. C. Röper-Kelmayr, D. Flöry, M. Guger, F. A. Fellner; Linz/AT magnetic resonance, subacute combined degeneration, vitamin B12 deficiency /ecr2010/C-3386 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 8

2 Learning objectives 1. To know the etiology, symptoms, and clinical value of vitamin B12 deficiency. 2. To know MR appearance of vitamin B12 deficiency induced myelopathy. 3. To know MR imaging strategies for evaluation and follow-up of this entity. Background Vitamin B12 deficiency has a broad spectrum of symptoms with effect on the brain, optic nerves, peripheral nerves, and especially also the spinal cord [1-4]. In the Western world it is a rare disease. Possible causes are: inadequate intake (most often seen in strict vegetarians), more often are malabsorption syndromes (bacterial overgrowth of the small bowel, pernicious anemia, regional enteritis, tropical sprue, surgical procedures, such as gastric fundus or ileal resection). Spectrum of clinical presentation: Initial symptoms are normally paresthesia in hands and feet, progressing to gait ataxia, sensory loss, distal weakness, especially concerning the legs. Untreated, the disease will progress to ataxic paraplegia. Furthermore, loss of vibratory and joint position sense, weakness, spasticity, hyperreflexia, may occur. Subacute combined degeneration: The terminus for the specific spinal cord lesions in vitamin B12 deficiency is subacute combined degeneration (SCD). Histologically, spongiform changes with foci of myelin and axonal destruction, especially in the posterior and lateral columns, are found. Rarely, also the anterior columns may be affected in an advanced stage. If therapy is started early enough, the patients will have full recovery. Otherwise, SCD becomes irreversible with permanent deficits. Differential diagnoses: Degeneration, demyelination, infection (e.g. tabes dorsalis), inflammatory and autoimmune (e.g. Grave's) disease, neoplasms (e.g. lymphoma), Friedreich's ataxia, sensory neuropathies and neuronopathies, other nutritional (e.g. vitamin E deficiency), and finally vascular disorders. In the past six years four patients with suspicion vitamin B12 deficiency underwent MR imaging (1.5 T) of the spinal cord in our institution using the following protocol: Page 2 of 8

3 Sagittal T2 TSE, sagittal T1 TSE pre and post (with fat saturation) gadolinium, axial T2 TSE, T1 TSE pre and post gadolinium. Imaging findings OR Procedure details MR findings in SCD may be variable. The leading feature is increased signal within the cervical and thoracic spinal cord concerning the posterior and lateral columns [1-4]. Some papers report that thickening of the cervical cord may be found on T1-weighted images. Furthermore, enhancement of the posterior and lateral columns can be seen on gadolinium-enhanced T1-weighted sequences. Normally, it is not seen. Involvement of the anterior columns (T2-weighted images) has been described only in one case report up to now [3]. If therapy begins early enough, complete recovery of the clinical symptoms is found in most of the cases. According to this fact, normalisation of the myelon signal on T2-weighted images is observed on the follow-up MR examinations. Therapy of SCD means monthly intramuscular injections of vitamin B12 for the whole life. The figures show a 23-year-old male patient who was admitted to our hospital with parasthesias of hand and feet, ataxia, urinary retention, and motory deficits of the extremities. In the patient's history there was ileal resection (due to volvulus). Serum levels of Vitamin B12 were normal. However, it is well-known that normal Vitamin B12 levels can be found in patients with SCD, e.g. with inadequate intake etiology of this disease. After substitution with vitamin B12 there was nearly complete remission of the symptoms and the pathological imaging findings within two weeks. Images for this section: Page 3 of 8

4 Fig. 1: 23-year-old patient with subacute combined degeneration (SCD) involving the cervical and thoracic cord. T2-weighted sagittal images from the cervical spinal cord before and after substitution with vitamin B12. After therapy significant remission of the spinal cord hyperintensities. Page 4 of 8

5 Fig. 2: 23-year-old patient with subacute combined degeneration (SCD) involving the cervical and thoracic cord. T2-weighted transverse images from the cervical spinal cord before and after substitution with vitamin B12. After therapy significant remission of the spinal cord hyperintensities. Fig. 3: Gadolinium-enhanced T1-weighted images. No pathological enhancement initially and in the follow-up examination. Page 5 of 8

6 Fig. 4: 23-year-old patient with subacute combined degeneration (SCD) involving the cervical and thoracic cord. T2-weighted sagittal images from the thoracic spinal cord before and after substitution with vitamin B12. After therapy significant remission of the spinal cord hyperintensities. Page 6 of 8

7 Fig. 5: 23-year-old patient with subacute combined degeneration (SCD) involving the cervical and thoracic cord. T2-weighted transverse images from the thoracic spinal cord before and after substitution with vitamin B12. After therapy significant remission of the spinal cord hyperintensities. Page 7 of 8

8 Conclusion Cave: Clinical, electrophysiological, and MR findings in subacute combined degeneration (SCD) often are diverse. Therefore, vitamin B12 deficiency should be considered in the differential diagnosis of all unclear spinal cord, peripheral nerve, and neuropsychiatrc disorders [4]. Principally, full recovery is likely, if therapy is started early enough. Delay in the diagnosis and therefore initation of therapy may result in permanent irreversible injury to the spinal cord [2]. Personal Information References 1. Ravina B, Loevner LA, Bank W. MR findigns in subacute combined degeneration of the spinal cord: a case of reversibile cervical myelopathy. AJR Am J Roentgenol 2000; 174: Pittock SJ, Payne3 TA, Harper CM. Reversible myelopathy in a 34-year-old man with vitamin B12 deficiency. Mayo Clin Proc 2002; 77: Karantanas AH, Markonis A, Bisbiyiannis G. Subacute combined degeneration of the spinal cord with involvement of the anterior columns: a new MRI finding. Neuroradiology 2000; 42: Hemmer B, Glocker FX, Schumacher M, Deuschl G, Lücking CH. Subacute combined degeneration: clinical, electrophysiological, and magnetic resonance imaging findings. J Neurol Neurosurg Psychiatry 1998; 65: Page 8 of 8