Astrocyte

LETTER TO EDITOR
Year
: 2014  |  Volume : 1  |  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

Correspondence Address:
Sourya Acharya
Department of Medicine, JN Medical College, DMIMS University, Sawangi (Meghe), Wardha - 442 004, Maharashtra
India




How to cite this article:
Acharya S, Shukla S, Banode P. Multiple spinal neurofibromatosis.Astrocyte 2014;1:250-251


How to cite this URL:
Acharya S, Shukla S, Banode P. Multiple spinal neurofibromatosis. Astrocyte [serial online] 2014 [cited 2020 Aug 5 ];1:250-251
Available from: http://www.astrocyte.in/text.asp?2014/1/3/250/157778


Full Text

Sir,

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}{Figure 2}{Figure 3}

 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. [1] 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. [2],[3]

References

1Thakkar SD, Feigen U, Mautner VF. Spinal tumours in neurofibromatosis type 1 : An MRI study of frequency, multiplicity and variety. Neuroradiology 1999;41:625-9.
2Créange A, Zeller J, Rostaing-Rigattieri S, Brugières P, Degos JD, Revuz J, et al. Neurological complications of neurofibromatosis type 1 in adulthood. Brain 1999;122 (Pt 3):473-81.
3Barber DB, Quattrone BE, Lomba ME, Able AC. Neurofibromatosis : An unusual cause of cervical myopathy. J Spinal Cord Med 1998;21:148-50.