High-dose chemotherapy and autologous stem cell transplantation in primary lymphomatoid granulomatosis of the central nervous system.

Primary lymphomatoid granulomatosis of the CNS (CNS-LG) is a rare lymphoid neoplasia associated Epstein-Barr Virus (EBV) and often accompanied by immunodeficiencies. No treatment standards have been defined yet. However, due to often devastating neurologic sequelae and based on similarities to diffuse large B-cell lymphoma, curative treatment requires intensive therapy protocols resembling protocols applied in CNS lymphoma. Here, the clinical courses and treatments of four primary CNS-LG patients in analogy to aggressive CNS-lymphomas including methotrexate, thiotepa, cytarabine, carmustine, and rituximab are presented. This is the first report on high-dose chemotherapy with CNS-directed drugs and autologous blood stem cell transplantation in primary CNS-LG.

Upper extremity nerve transfers for acute flaccid myelitis: a systematic literature review.

BACKGROUND: Acute flaccid myelitis (AFM) is a rare disease that commonly affects young children. AFM's pathophysiology involves loss of lower motor neurons following a viral infection and induces acute asymmetric flaccid paralysis most commonly in the upper extremities. Nerve transfers have emerged as a treatment option for these patients with permanent motor deficits. OBJECTIVE: To summarize the literature and report safety and efficacy outcomes following nerve transfers for recovery of shoulder abduction and external rotation, and elbow flexion and extension in pediatric patients with AFM. Recovery of at least antigravity function was defined as a successful outcome. This systematic review was performed according to the PRISMA guidelines. The PubMed, Embase and Cochrane databases were utilized. RESULTS: Five studies comprising 44 patients (median age 2.95 years; 71% male), and 93 upper extremity nerve transfers were included. Thirty-eight patients received 65 nerve transfer procedures aiming for recovery of shoulder abduction and/or external rotation with a transfer to the axillary and/or suprascapular nerve. The recovery of shoulder abduction and external rotation was achieved in 40.7% (n = 11/27) and 60% (n = 6/10) of patients, respectively. Time from injury to surgery showed an inverse relationship with the odds for successful recovery (OR: 0.81; 95% CI: 0.64-1.02; p = 0.07); however, statistical significance was not reached. Successful recovery of elbow flexion with a transfer to the musculocutaneous was reported at a rate of 92.3% (n = 12/13). Successful re-innervation of the radial nerve with recovery of elbow extension was found in 75% (n = 6/8) of patients. No complications were reported. CONCLUSIONS: Upper extremity nerve transfers appear to be promising and safe for AFM patients. Shoulder abduction is the most challenging upper extremity function to recover. Further studies are warranted to identify whether nerve transfers are associated with superior outcomes when performed earlier.

Recommendations for Therapy following Nerve Transfer for Children with Acute Flaccid Myelitis.

AIM: To provide recommendations for pre- and post-operative occupational and physical therapy for children with acute flaccid myelitis (AFM). METHODS: Writing panel members consisted of an interdisciplinary team of seven healthcare professionals specializing in the care of children with AFM. The panel reviewed background material on AFM, nerve transfer, and rehabilitation principles applied to pediatrics. Recommendations were prioritized if evidence was available. Where there was no known evidence to support a recommendation, this was noted. RECOMMENDATIONS: Communication and coordination among interprofessional team members are vital to a comprehensive family-centered rehabilitation program. Surgical planning should include team preparation accounting for frequency, duration, and timing of treatment, as well as individual characteristics and developmental status of the child. Recommendations for pre-operative and six phases of post-operative therapy address assessment, strengthening, range of motion, orthoses, performance of functional activity, and support of the family. CONCLUSION: Rehabilitation following nerve transfer in children with AFM requires interdisciplinary collaboration and a multisystem approach to assessment and treatment. As new evidence becomes available, recommendations may be revised or replaced accordingly.

The Utilization of Nerve Transfer for Reestablishing Shoulder Function in the Setting of Acute Flaccid Myelitis: A Single-Institution Review.

BACKGROUND: Acute flaccid myelitis (AFM) is a rare disease of young children. The typical presentation involves acute-onset flaccid paralysis in one or more extremities with a nonspecific viral prodrome. Long-term outcomes demonstrate that functional recovery plateaus around six to nine months. The purpose of this study was to evaluate the efficacy of nerve transfers for restoring shoulder function in these patients. METHODS: A retrospective review of all patients diagnosed with AFM at a single institution. Shoulder function was evaluated using the active movement scale (AMS). Children at a minimum of six months after diagnosis with plateaued shoulder AMS scores of 4 or less were indicated for surgery. RESULTS: Eleven patients were identified with a mean time from symptom onset to surgery of 12 months. Average follow-up was 19 months. The mean AMS score at follow-up for shoulder external rotation and abduction was 4.6 and 2.8, respectively. A total of six different nerve transfers with five different donor nerves were used individually or in conjunction with each other. The most common transfers were from the spinal accessory nerve to the suprascapular nerve (n = 8) and from the intercostal nerves ×3 to the axillary nerve (n = 5). Patients who received a transfer from the radial nerve to the axillary nerve (n = 2) had the best functional returns, with the mean AMS score of 6.5 in both external rotation and abduction at follow-up. CONCLUSION: Nerve transfer procedures may help restore shoulder function in the setting of AFM. Combination procedures that involve a transfer from the radial nerve to the axillary nerve may provide the best functional results.

Contralateral Obturator Nerve to Femoral Nerve Transfer for Restoration of Knee Extension After Acute Flaccid Myelitis: A Case Report.

CASE: A 7-year-old boy presented with left femoral and obturator nerves (ONs) palsy after an asthmatic attack with a viral prodrome, and his right lower limb was unaffected. He was diagnosed with acute flaccid myelitis (AFM) after positive spinal magnetic resonance imaging findings. After contralateral ON to femoral nerve transfer (CONFNT), his left quadriceps was reinnervated at 5.5 months, full knee extension was recovered at 14 months, and good functional outcomes were achieved at 31 months. CONCLUSIONS: This first clinical report on CONFNT demonstrated a feasible good alternative in treating young patients with AFM with unilateral L2-L4 palsy and short duration of deficit.

Early results of nerve transfers for restoring function in severe cases of acute flaccid myelitis.

OBJECTIVE: To describe early functional outcomes of nerve transfer surgery in a relatively large cohort of patients with acute flaccid myelitis (AFM). METHODS: A retrospective case analysis was made of patients with AFM treated with nerve transfer surgery between 2007 and 2018. Surgical criteria were persistent motor deficits after 6 months from onset and available donor nerves. Thirty-two patients with AFM were evaluated; 16 underwent nerve transfer surgeries. Motor function was evaluated by a licensed occupational therapist using the Active Movement Scale preoperatively and during follow-up examinations. Patients with 6 or more months of follow-up were included in the analysis. Patients with procedures other than nerve transfers were excluded. RESULTS: Sixteen patients with AFM had nerve transfers, with a male predominance (75%) and median age of 2.5 years (range = 4 months-12 years). Eleven patients had a minimum 6 months of follow-up. Nerve transfers to restore elbow function had 87% excellent recovery for elbow flexion and 67% for elbow extension. Finger and thumb extension were full against gravity in 1 patient (100%). Shoulder external rotation was excellent in 50% of patients and shoulder abduction in only 20%. Nine of 10 patients (90%) had resolution of shoulder pseudosubluxation following nerve transfer to the suprascapular nerve. INTERPRETATION: Patients with AFM with persistent motor deficits 6 to 9 months after onset benefit from nerve transfer surgery. Restoration of elbow function was more reliable than restoration of shoulder function. We recommend early referral of patients with incomplete recovery to a center experienced in nerve transfers for timely evaluation and treatment. ANN NEUROL 2019;86:607-615.