Approximately 100 cases of segmental neurofibromatosis (NF5) have been reported in the recent literature. Patients with NF5 present with café-au-lait macules, freckles, and/or neurofibromas limited to one or adjacent dermatomes. Neurofibromas arising in NF5 have been uniformly considered to be benign; patients were thought to have an excellent prognosis without the risk of developing malignant peripheral nerve sheath tumors (PNSTs), which are characteristic in patients with the generalized form of this disease, von Recklinghausen's NF. In this report the authors detail the first observations of malignant PNSTs in two patients with NF5. Indications for surgical removal of a neurofibroma in a patient with NF include pain. neurological impairment, compression of adjacent structures, cosmetic disfigurement, and rapid tumor growth suggestive of malignant degeneration. Surgical indications are similar for patients with NF5. All patients with neurofibromas should be considered at risk for malignant degeneration.

We present the case of a patient with a rare trigeminal ganglion amyloidoma that closely mimicked idiopathic trigeminal neuralgia. Even in the absence of systemic signs of amyloidosis, this benign protein deposition disease should be considered in the differential for atypical dysesthesias of the trigeminal dermatomes. Furthermore, central and peripheral nervous system amyloidomas respond well to surgical resection and rarely recur.

Study Design. Pilot randomized controlled trial. Objective. To examine the acceptability and preliminary safety and outcome effects of an early self-directed home exercise program (HEP) performed within the first 6 weeks after anterior cervical discectomy and fusion (ACDF). Summary of Background Data. Little is known regarding optimal postoperative management after ACDF. Methods. Thirty patients (mean ± standard deviation, age = 50.6 ± 11.0 years, 16 women) undergoing ACDF were randomized to receive an early HEP (n = 15) or usual care (n = 15). The early HEP was a 6-week self-directed program with weekly supportive telephone calls to reduce pain and improve activity. Treatment acceptability was assessed after the intervention period (6 weeks after surgery). Safety (adverse events, radiographic fusion, revision surgery) was determined at routine postoperative visits. Disability (Neck Disability Index), pain intensity (Numeric Rating Scale for neck and arm pain), physical and mental health (SF-12), and opioid use were assessed preoperatively, and at 6 weeks and 6 and 12 months after surgery by an evaluator blinded to group assignment. Results. Participants reported high levels of acceptability and no serious adverse events with the early HEP. No difference in fusion rate was observed between groups (P > 0.05) and no participants underwent revision surgery. The early self-directed HEP group reported lower 6-week neck pain than the usual care group (F = 3.3, P = 0.04, r 2 = 0.3, mean difference = −1.7 [−3.4; −0.05]) and lower proportion of individuals (13% vs. 47%) using opioids at 12 months (P = 0.05). No other between-group outcome differences were observed (P > 0.05). Conclusion. An early self-directed HEP program was acceptable to patients and has the potential to be safely administered to patients immediately after ACDF. Benefits were noted for short-term neck pain and long-term opioid utilization. However, larger trials are needed to confirm safety with standardized and long-term radiograph assessment and treatment efficacy. Level of Evidence: 2

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