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SPINE IMAGE
Year : 2022  |  Volume : 9  |  Issue : 1  |  Page : 65-66

“Garland” of neurofibromas in neurofibromatosis type 1


1 Department of Neurosurgery, All India Institute of Medical Sciences, Delhi, India
2 Department of Neurology, All India Institute of Medical Sciences, Delhi, India

Date of Submission12-Dec-2021
Date of Acceptance15-Jan-2022
Date of Web Publication9-Mar-2022

Correspondence Address:
Sachin Anil Borkar
Room 717, 7th Floor, CN Centre, Department of Neurosurgery, All India Institute of Medical Sciences, Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/joss.joss_33_21

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How to cite this article:
Kamra D, Bansal T, Anand P, Borkar SA. “Garland” of neurofibromas in neurofibromatosis type 1. J Spinal Surg 2022;9:65-6

How to cite this URL:
Kamra D, Bansal T, Anand P, Borkar SA. “Garland” of neurofibromas in neurofibromatosis type 1. J Spinal Surg [serial online] 2022 [cited 2022 May 27];9:65-6. Available from: http://www.jossworld.org/text.asp?2022/9/1/65/339272



A 19-year-old adult male presented to the Neurosurgery Outpatient Department with a complaint of progressive weakness of all the four limbs since 2 years starting from difficulty in walking and then progressing to upper limbs. He had decreased grip in both hands and was unable to hold objects. He had complaints of urinary incontinence over the last 1 month. On physical examination, there were multiple small subcutaneous nodules. Sensations were decreased to all modalities below C4 level in upper and lower limbs (UL/LL), power in both was UL 4/5 and both it was LL 3/5. Tone was increased in both the lower limbs; deep tendon reflexes were exaggerated.

Magnetic resonance imaging demonstrated well-defined multilevel, bilateral symmetric mass lesions measuring approximately 1.5 cm × 0.9 cm, isointense on T1, and hyperintense on T2-weighted images, involving bilateral neural foramina of C3–C6 levels and causing their widening. It was giving an appearance of a “garland”as shown in [Figure 1]. The patient has been planned for surgical excision of symptomatic lesion and is awaiting surgery.
Figure 1: T1 Contrast and T2 weighted imaging of patient showing characteristic 'Garland' configuration of bilateral symmetrical mirror image neurofibromas.

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Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder which occurs due to mutation or deletion of NF1 gene.[1] NF1 is a multi-system disorder which has varied clinical presentations. Neurofibromas are considered a clinical hallmark of this disease. There is a tendency to form multiple neurofibromas however, very few cases have a symmetrical arrangement. Neurofibromas can be nodular or diffuse and are generally well demarcated. Neurofibromas usually grow along the involved nerve confined by epineurium. Many patients with paraspinal neurofibromas have a tendency to cause radiculo-myelopathy. Surgery is usually reserved for symptomatic lesions causing cord compression.[1],[2]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Rasmussen SA, Friedman JM. NF1 gene and neurofibromatosis 1. Am J Epidemiol 2000;151:33-40.  Back to cited text no. 1
    
2.
Gutmann DH, Ferner RE, Listernick RH, Korf BR, Wolters PL, Johnson KJ. Neurofibromatosis type 1. Nat Rev Dis Primers 2017;3:17004.  Back to cited text no. 2
    


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