Headache and neck pain are common ailments. What isn’t so common is the types of headaches that do not resolve with normal treatment, and reach the point where activities of daily life are routinely interrupted. Add that to the complication of an issue of neurofibromatosis and you have an interesting case indeed.
Neurofibromatosis is a group of three genetic disorders. These changes in genes predispose individuals to developing tumours in and along the nervous system. The three subtypes of neurofibromatosis are; neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and Schwannomatosis. NF1 is the most popular subtype and is characterised by benign tumour growth on nerve sheaths and the development of brown patches on the skin (cafe-au-lait spots). Tumour development in the central nervous system progressing through to the peripheral nervous system are also common.NF2 is characterised by benign and slow-growing tumours in both ears, causing hearing loss in some cases. Schwannomatosis is the rarest of the three, and can cause tumours to grow along the cranial, spinal, and peripheral nerves.
Musculoskeletal dysfunction is common in NF1 patients and often manifests as scoliosis, congenital pseudarthrosis, bone cysts, cortical bone thinning, and subperiosteal bone hyperplasia. Standard care for NF1 patients includes annual physical examinations and the management of symptoms. Physiotherapy and chiropractic are commonly sought for musculoskeletal pain relief. These and other alternative therapies have been found to be effective in providing some pain relief (1)
A previous case published in the Journal of the Canadian Chiropractic Association covered a similar case with a 21-year-old female NF1 patient. The patient presented for chiropractic care with headaches, and neck, face, and low back pain. This patient had a history of surgeries related to her condition, unlike the current case, but similarly found pain relief concomitant chiropractic care. (2)
The present case
A new case has just emerged in which a 21-year-old male with type 1 neurofibromatosis presented for chiropractic care for the predominant complaints of neck pain and headaches. He had been diagnosed with NF1 by a dermatologist some 10 years prior. His neck and head pain had persisted for 6 months, and during examination the intensity of the pain was rated a 2/10 and an 8/10 respectively. The pain was commonly on the side of the head and neck and was described by the patient as a ‘pulsating squeezing’ around the right forehead.
The pain would last about an hour and would occur approximately 2-3 per day, unsurprisingly disturbing sleep, and daily activities. He had no history of trauma or other neurological disorders and had a prior negative brain MRI and neurological assessment of the headache. The patient did receive a coincidental diagnosis of attention deficit disorder and learning disability by a clinical psychologist.
In attempts to achieve some relief from the headache, which was diagnosed as a chronic headache by his primary care physician, he had tried non-steroidal anti-inflammatory drugs, anticonvulsants, narcotics, physical therapy, acupuncture, and psychotherapy but with no success. Analgesics were the only source of relief the patient was relying on.
In addition to the pain, the patient was also concerned about the painless bumps, neurofibromas, on the skin.
During the examination it was found the patient has a restricted neck range of motion and a loss of cervical lordosis. There were several flat, light brown spots (cafe-au-lait spots) on the skin across the back. Multiple soft, nodular lesions (cutaneous neurofibromas) were also on the back and both arms. Both of these observations are expected in NF1 patients.
Physical examination revealed tenderness and hypertonicity of cervical extensor muscles on both sides, and a restricted range of motion to 40 deg at cervical extension and 50 deg at rotations (with the normal range >60 deg and >80 deg respectively)
Spinal palpation revealed dysfunction between spinal segments at C2/3, C4/5, C5/6, T1/2, T3/4, T7/8. Eye movement, cerebellar, neurological, orthopedic, and walking function assessments were normal. Neck Disability Index (NDI) was rated at 84%, indicating a high level of disability. Radiographic assessment of the cervical spine revealed the reduced cervical lordosis with Cobb angle at 3 degrees. The patient was diagnosed by the chiropractor as having a cervical syndrome plus secondary headache.
What was done?
The aims of care were to reduce pain, muscle hypertonicity, and restore spinal mobility. The patient received care daily for the first 6 days and, following a re-evaluation the frequency of care, was reduced to 3 sessions a week for 4 weeks. Following those 4 weeks, care was again reduced to 2 sessions a week for another 8 weeks. After the first 13 weeks, the patient was seen monthly.
Chiropractic care consisted of thermal ultrasound therapy, cervical manipulation with a high-velocity, low-amplitude force, and skin-to-skin contact manual adjustment. After the first 6 days of care the patient reported a reduction in pain severity of his headache, now rating it a 5/10. During the next stage of care the patient reported a decrease in frequency and duration of his headache, improvement in sleep quality, and a reduction in his reliance on analgesics.
During the maintenance phase of care, monthly sessions, the management was supplemented with at-home posture correction exercises. The patient only followed this recommendation for 1 week, as he had a busy work schedule. The patient continued asymptomatic for 12 months.
At this stage the patient took a short break from care of about 3 months. His headaches quickly returned but were resolved immediately after 4 sessions of care across 2 weeks. During the third year under chiropractic care a radiographic re-evaluation was completed and revealed a change in cervical lordosis from Cobb angle at 3 degrees to now 10 degrees – a significant improvement.
The patient continued with ongoing monthly sessions and remained symptom free. During the fifth year under care another evaluation was completed, revealing a cervical lordosis Cobb angle of 20 deg. However, cutaneous neurofibromas had increased in both size and number during this time.
This case is exciting as it details the role spinal manipulation may be able to play in improving neck pain and spinal deformity in NF1 patients. This case is especially interesting as the patient had sought many different modes of care, deriving little success in pain relief, before opting for chiropractic intervention.
The author of the current case proposes the mechanisms behind the improvements observed may include correcting spinal dysfunction, breaking-up joint adhesions, mobilising restricted tissues, facilitation of trunk muscle strength, and possibly releasing the nerve entrapped by limited movement. They also note that supporting the autonomic nervous system, which when dysfunctional can contribute to tension type headaches, may also be relevant to this case.
As previously mentioned, manipulation of the spine in NF1 patients should be approached with great caution. Radiographic evaluation before beginning treatment, and at regular intervals throughout care is recommended.
While the normal case report limitations apply in that we cannot generalise these findings to the wider population, the predominant limitation of this report is that the cause of the neck pain and headache were unknown. We do know though, that as the autonomic nervous system was supported and improvements were recognised in mobility, adhesions, nerve issues and areas of restriction, the headaches which had refused to budge under other treatments “coincidentally” disappeared.
We find that very interesting.
- F Buono et al. Pain symptomology, functional impact, and treatment of people with Neurofibromatosis type 1. Journal of Pain Research. 2019. 12(1); 2555-2561
- J Mignelli. L Tollefson. E Stefanowicz. Conservative management of neck and thoracic pain in an adult with neurofibromatosis-1. J Can Chiropr Assoc. 2021. 65(1); 121-127
- E Chun-Pu Chu et al. Remission of headache and neck pain following chiropractic manipulative treatment in a patient with neurofibromatosis. Chiropractic Journal of Australia. 2022.