A 19 y/o man with limb paresis after MVA

 

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Doctor's Information

Name : Morteza
Family : Sanei Taheri
Affiliation :Radiology Department,Shohada Tajrish Hospital,SBMU
Academic Degree : Associate Professor of Radiology
Email : This email address is being protected from spambots. You need JavaScript enabled to view it.
Resident : maryam babaie

 

Case Section

Neuroradiology

 

Patient's Information

Gender : Male
Age : 19

 

Clinical Summary

A 19 y/o man with limb paresis after MVA.

 

Imaging Findings

Selected images from MRI of the spine demonstrate innumerable nodules located on the skin, in the soft tissues and along nerve roots. These are intermediate signal on T1 with moderate contrast enhancement, and are high signal on T2. There is marked cord compression at C2-C3 levels.Multiple plexiform neurofibroma are seen in the majority of cervico-dorso-lumbar spine and pelvis. lateral meningocele is seen in thoracic spine.

 

Differential Diagnosis

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Final Diagnosis

Neurofibromatosis type 1

 

Discussion (Related Text)

Neurofibromatosis type 1 (NF1) or von Recklinghausen disease is a multisystem neurocutaneous disorder and the most common phakomatosis . To make the clinical diagnosis you require two or more of following : cafe au lait spots evident during 1 year , two or more neurofibromas or one plexiform neurofibroma , optic nerve glioma , distinctive osseous lesion ,sphenoid wing dysplasia , two or more iris hamartomas (Lisch nodules( , axillary or inguinal freckling , primary relative with NF 1 .MRI allows noninvasive identification of intradural neurofibromas and helps determine their relationship to the neural foramina, canal, and thecal sac spinal cord. The target sign (bright on T2, with dark collagen centrally) is highly suggestive of a peripheral neurofibroma on MRI. It may be seen with plexiform neurofibromas as well. Paraspinal neurofibromas may be dumbbell shaped, fusiform, or spherical. They are slightly hyperintense relative to muscle on T1-weighted images, with a hyperintense periphery and a hypointense core on T2-weighted images. A lateral thoracic meningocele, which is known to be associated with NF1, is seen as a CSF-filled sac extending through a widened neural foramina.

 

References

Nawaz Khan a ,et al . Neurofibromatosis Type 1 Imaging. Medscape. Updated: Jun 19, 2013

 

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