Transverse myelitis and neuralgic amyotrophy

Neuralgic amyotrophy (NA), a lower motor neuron (LMN) lesion, presents a flaccid monoplegia of the upper extremity in children. Transverse myelitis (TM) may present with either an LMN or an upper motor neuron (UMN) injury, depending on areas affected on the cord. Both entities are inflammatory and autoimmune in nature. Diagnosis is with MRI and serological studies as well as nerve conduction studies (NCS) and electromyography (EMG). Treatment varies slightly, but includes immunosuppression with steroids, replacement of antibodies with plasmapheresis in addition to intravenous immunoglobulin (IVIG), immunosuppressant, and antineoplastic agents. Further management includes rehabilitation measures with stretching, strengthening, range of motion, neuromuscular electrical stimulation, patient and family education, and equipment evaluation. Children with persistent LMN deficits and limited recovery (6–8 months after onset of disease) may warrant surgical considerations for peripheral nerve surgery. This may include the use of nerve transfers. Secondary surgery, including muscle and tendon transfers, can be considered 1–2 years after disease onset, if persistent residual deficits are present. Most children with NA have good outcomes with resolution of symptoms and improvement in function. Children with TM have a less favorable outcome with one third resolution rate.