What is DNET (Diffuse Neurofibroma)

From the Guidelines

Adults with Neurofibromatosis Type 1 (NF1) should be managed with a comprehensive approach that prioritizes their quality of life, considering the potential for chronic pain, sleep disturbances, and other complications. When managing NF1, it is essential to consider the potential for various complications, including Parkinson’s disease and multiple sclerosis, as suggested by a study published in Genetics in Medicine 1. Key aspects of NF1 management include:

• Monitoring for sleep disturbances, as a prospective questionnaire study found a high frequency of sleep disturbance, impaired sleep, and excessive daytime sleepiness in adults with NF1 1
• Evaluating and managing chronic pain, which can be nociceptive or neuropathic and may arise from various sources, including pseudarthrosis, scoliosis, or peripheral neuropathy 1
• Considering the potential for NF1 neuropathy, a rare, adult-onset, non-progressive, symmetrical polyneuropathy that features a typically indolent, nerve-length-dependent sensorimotor polyneuropathy with predominantly sensory signs 1
• Suspecting glomus tumors in patients with localized tenderness, severe paroxysmal pain, and sensitivity to cold, and using MRI imaging for diagnosis 1
• Being aware of the potential for malignant peripheral nerve sheath tumors (MPNST) in patients with new-onset, increasingly severe pain 1. In terms of eligibility to serve in the armed forces, a history of neurofibromatosis is a disqualification for enlistment in the United States, as noted in Army Regulation 40-501 1.

From the Research

Dnet and Acute Mountain Sickness

• Dexamethasone has been studied as a potential treatment and preventative measure for acute mountain sickness (AMS) in several studies 2, 3, 4, 5, 6
• The evidence suggests that dexamethasone can effectively reduce the symptoms of AMS when given prophylactically or as a treatment 2, 3, 5
• A systematic review and meta-analysis found that dexamethasone could reduce the incidence of AMS with an odds ratio of 6.03 (95% CI, 2.23 to 21.00) for dexamethasone compared with placebo 6

Dexamethasone Dosage and Efficacy

• Different doses of dexamethasone have been studied, including 2 mg, 4 mg, 8 mg, 12 mg, and 16 mg per day 3, 4, 6
• The evidence suggests that higher doses of dexamethasone (4 mg or 8 mg per day) are more effective in preventing and treating AMS than lower doses (2 mg per day) 3, 4
• However, the optimal dose and duration of dexamethasone treatment for AMS are still unclear and require further study 6

Mechanism of Action and Side Effects

• Dexamethasone is thought to work by reducing cerebral edema, which is a key component of AMS 2, 5
• However, dexamethasone has also been shown to have no effect on fluid shifts, oxygenation, sleep apnea, urinary catecholamine levels, and other physiological parameters in some studies 2
• Common side effects of dexamethasone include mild hyperglycemia, and there is a risk of adrenal cortical suppression with long-term use 2, 4