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| SPINE IMAGE |
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| Year : 2022 | Volume
: 9
| Issue : 1 | Page : 65-66 |
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“Garland” of neurofibromas in neurofibromatosis type 1
Devasheesh Kamra1, Tungish Bansal1, Pooja Anand2, Sachin Anil Borkar1
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 Submission | 12-Dec-2021 |
| Date of Acceptance | 15-Jan-2022 |
| Date of Web Publication | 9-Mar-2022 |
Correspondence Address:
Sachin Anil Borkar
Room 717, 7th Floor, CN Centre, Department of Neurosurgery, All India Institute of Medical Sciences, Delhi
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/joss.joss_33_21
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 |
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 | |  |
| 1. | Rasmussen SA, Friedman JM. NF1 gene and neurofibromatosis 1. Am J Epidemiol 2000;151:33-40.  |
| 2. | Gutmann DH, Ferner RE, Listernick RH, Korf BR, Wolters PL, Johnson KJ. Neurofibromatosis type 1. Nat Rev Dis Primers 2017;3:17004. |
[Figure 1]
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