|LETTER TO EDITOR
|Year : 2014 | Volume
| Issue : 3 | Page : 250-251
Multiple spinal neurofibromatosis
Sourya Acharya1, Samarth Shukla2, Pankaj Banode3
1 Department of Medicine, JN Medical College, DMIMS University, Sawangi (Meghe), Wardha, Maharashtra, India
2 Department of Pathology, JN Medical College, DMIMS University, Sawangi (Meghe), Wardha, Maharashtra, India
3 Department of Radiodiagnosis, JN Medical College, DMIMS University, Sawangi (Meghe), Wardha, Maharashtra, India
|Date of Web Publication||27-May-2015|
Department of Medicine, JN Medical College, DMIMS University, Sawangi (Meghe), Wardha - 442 004, Maharashtra
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Acharya S, Shukla S, Banode P. Multiple spinal neurofibromatosis. Astrocyte 2014;1:250-1
A 54-year-old male presented to us with a history of pain in neck and back on and off since 2 years. Pain was dull aching type and aggravated with movements. There was no history of radiation, weakness in arms, hands and legs, tingling in extremities, thinning of the limbs, and gait disturbances. Bowel and bladder habits were normal.
His general and systemic examination was normal. Examination of the neck revealed slight restriction in neck extension. Straight leg raise (SLR), reverse SLR tests were negative. Deep tendon reflexes were normal, and plantars were bilateral flexors. There were no sensory deficits. Dermatological examination revealed axillary freckling. Eight cafe au lait macules were present on the back, abdomen, arms, and legs, varying between 1.5, 4, and 5 cm subcutaneous nodular lesions of millimeter size in the both arm and forearm on region. Lisch nodules were not detected on slit lamp examination of the eye.
Magnetic resonance imaging (MRI) of the cervical and lumbosacral cord revealed multiple neurofibromatosis of the spine [Figure 1], [Figure 2] and [Figure 3].
|Figure 1: Magnetic resonance imaging shows bilateral fusiform enlargement of nerves in the cervical region|
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|Figure 2: Contrast magnetic resonance imaging cervical spine scan reveals homogenous enhancement of enlarged nerve roots|
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|Figure 3: Contrast magnetic resonance imaging (MRI) lumbar spine scan reveals homogenous enhancement of enlarged nerve roots. MRI scan reveals bilateral symmetrical nodular and fusiform mild homogenous enhancing enlargement of peripheral nerves throughout the whole spine with involvement of nerve roots. Intradural nodular component also noted in the cervical region. Above MRI findings are s/o the plexiform neurofibromatosis|
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| Discussion|| |
Neurofibromatosis type 1 (NF1) is a common autosomal dominant disorder. The affected individuals develop both benign and malignant tumors. Neurofibromas are benign tumors arising from the endoneurium and are characteristic of NF1. They develop as discrete focal cutaneous or subcutaneous tumors or more diffuse plexiform neurofibromas that grow along the length of nerves frequently involving multiple nerve fascicles, branches and plexuses. Spinal nerve root neurofibromas can arise throughout the spine and at multiple levels, and likely represent plexiform tumors based on the involvement of multiple nerve fascicles. A neuroimaging study detected cervical nerve root tumors in 52% of 54 patients, but none developed symptoms of cord compression.  There are infrequent single case reports and limited information in the literature about clinical presentation, management and prognosis of patients with NF1 with cervical or lumbar cord compression from plexiform neurofibromas. ,
| References|| |
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Barber DB, Quattrone BE, Lomba ME, Able AC. Neurofibromatosis : An unusual cause of cervical myopathy. J Spinal Cord Med 1998;21:148-50.
[Figure 1], [Figure 2], [Figure 3]