Subscribe to RSS
DOI: 10.1055/s-0030-1249654
© Georg Thieme Verlag KG Stuttgart · New York
Enhancing Cranial Nerves and Cauda Equina: An Emerging Magnetic Resonance Imaging Pattern in Metachromatic Leukodystrophy and Krabbe Disease
Publication History
received 14.11.2009
accepted 17.02.2010
Publication Date:
05 May 2010 (online)
![](https://www.thieme-connect.de/media/neuropediatrics/200906/lookinside/thumbnails/10.1055-s-0030-1249654-1.jpg)
Abstract
We report on three cases of infantile Krabbe disease and one case of infantile metachromatic leukodystrophy showing magnetic resonance (MR) imaging findings of diffuse and coexistent cranial nerve and cauda equina nerve roots enhancement. Such findings may be simultaneous, or even precede, typical white matter abnormalities and, in the appropriate clinical context, may facilitate an earlier diagnosis. There is a rational for the use of contrast agents and craniospinal MR imaging during the first imaging of children with a history of psychomotor regression and clinical evidence of peripheral nerve involvement to exclude differential diagnoses.
Key words
MRI - inborn errors of metabolism - peripheral nervous system
References
- 1 Bernal OG, Lenn N. Multiple cranial nerve enhancement in early infantile Krabbe's disease. Neurology. 2000; 54 2348-2349
- 2 Berns DH, Blasser S, Ross JS. MR imaging with GdDTPA in leptomeningeal spread of lymphoma. J Comput Assist Tomogr. 1988; 12 494-500
- 3 Beslow LA, Schwartz ES, Bönnemann CG. Thickening and enhancement of multiple cranial nerves in conjunction with cystic white matter lesions in early infantile Krabbe disease. Pediatr Radiol. 2008; 38 694-696
- 4 Cellerini M, Salti S, Desideri V. et al . MR imaging of the cauda equina in hereditary motor sensory neuropathies: correlations with sural nerve biopsy. AJNR Am J Neuroradiol. 2000; 21 1793-1798
- 5 Crino PB, Zimmerman R, Laskowitz D. et al . Magnetic resonance imaging of the cauda equina in Guillain-Barré syndrome. Neurology. 1994; 44 1334-1336
- 6 Dayan AD. Peripheral neuropathy of metachromatic leukodystrophy: observations on segmental demyelination and remyelination and intracellular distribution of sulphatide. J Neurol Neurosurg Psychiatry. 1967; 30 311-314
- 7 Ganesan K, Desai S, Hegde A. Multiple cranial nerve enhancement: uncommon imaging finding in early infantile Krabbe's disease. J Neuroimaging. 2008; 20 1-3
- 8 Given II CA, Santos CC, Durden DD. Intracranial and spinal MR imaging findings associated with Krabbe's disease: case report. AJNR Am J Neuroradiol. 2001; 22 1782-1785
- 9 Maia Jr AC, da Rocha AJ, da Silva CJ, Rosemberg S. Multiple cranial nerve enhancement: a new MR imaging finding in metachromatic leukodystrophy. AJNR Am J Neuroradiol. 2007; 28 999
- 10 Mantienne C, Albucher JF, Catalaa I. et al . MRI in Lyme disease of the spinal cord. Neuroradiology. 2001; 43 485-488
- 11 Moser HW. Peripheral nerve involvement in Krabbe disease: a guide to therapy selection and evaluation. Neurology. 2006; 67 201-202
- 12 Singh RK, Leshner RT, Kadom N. et al . Isolated cranial nerve enhancement in metachromatic leukodystrophy. Pediatr Neurol.. 2009; 40 380-382
- 13 Toldo I, Carollo C, Battistella PA. et al . Spinal cord and cauda equina MRI findings in metachromatic leukodystrophy: case report. Neuroradiology. 2005; 47 572-575
- 14 van der Knaap MS, Valk J. Magnetic Resonance of Myelination and Myelin Disorders, 3rd edn.. Berlin: Springer; 2005
- 15 Vasconcellos E, Smith M. MRI nerve root enhancement in Krabbe disease. Pediatr Neurol. 1998; 19 151-152
Correspondence
Giovanni Morana
Department of Neuroradiology
G. Gaslini Children's Research
Institute
Largo G Gaslini 5
16147 Genoa
Italy
Phone: +39/010/563 6516
Fax: +39/010/377 9798
Email: giovannimorana@ospedale-gaslini.ge.it