Age: 41.0
Sex: F
Vaccine: FLUX, FLUX
Symptoms: Areflexia, Bone scan normal, CSF pressure increased, CSF protein increased, CSF shunt operation
Clinical narrative: This medically confirmed literature report (initial receipt 23-Jun-2015) concerns a 41 year-old female patient who had a history of an uncomplicated spinal hemangioma resection. On an unspecified date, the patient received Influenza virus vaccine (manufacturer and batch number not provided). On an unspecified date, within 1 week of receiving the influenza vaccine, the patient developed bilateral numbness and paresthesia in her hands and feet. Her exam was notable for distal lower extremity weakness and sensory loss and absent ankle reflexes. A lumbar puncture demonstrated a modestly elevated protein level of 0.55 g/L but an otherwise normal cerebrospinal fluid (CSF) profile. Nerve conduction studies demonstrated features that were suggestive of an acquired demyelinating polyneuropathy (prolonged F-wave latencies, slowed median and ulnar velocities, a partial conduction block along the median and ulnar nerves) and axonal loss (active denervation and no motor unit potentials in the tibialis anterior and medial gastrocnemius). She was diagnosed with Guillain-Barre syndrome and treated with multiple rounds of intravenous immunoglobulin followed by mycophenolate mofetil. Despite treatment, she had persistent lower extremity weakness along with sensory loss in her bilateral lower extremities and hands. A repeat CSF examination was notable for protein of 1.15 g/L. A serum protein electrophoresis study demonstrated elevated free lambda light chain (31) with an abnormal banding pattern. Subsequent immunofixation confirmed monoclonal gammopathy with a dual immunoglobulin A (IgA) M-spike and two IgA lambda M components of 0.15 g/dl and 0.06 g/dl, respectively, in the beta region. These findings were thought to be most consistent with chronic inflammatory demyelinating polyneuropathy (CIDP). Given her worsening polyneuropathy, she underwent an MRI of the spinal cord which demonstrated a 2.1x1, 4x3.6 cm homogeneously enhancing mass in the right T12/L1 neural foramen with expansion into the right epidural space. She underwent a T12 laminectomy for reaction of the extradural mass and the pathology demonstrated a spinal hemangioma. She did not experience resolution of her symptoms following surgery. Approximately 6 months after the initial onset of symptoms, she reported a severe bifrontal headache, blurry vision, diplopia, and pulsatile tinnitus. A repeat lumbar puncture demonstrated an opening pressure of 31 cm H2O. Imaging of the brain including CT (computerised tomography) scans, MRI (magnetic resonance imaging), and magnetic resonance venogram was unremarkable. Given her complaint of visual deterioration, an ophthalmologic exam was pursued which demonstrated bilateral papilledema and visual acuity of 20/20 oculus dexter (OD) and 20/30 oculus sinister (OS). Her prednisone dose was increased and she was started on acetazolamide. She was subsequently admitted to the neurology service for workup of her persistent headaches, progressive visual deterioration, and papilledema. Over 2 weeks, her visual acuity decreased to 20/70 OD and 20/60 OS, with continued papilledema. Her symptoms initially improved after an additional lumbar puncture (opening pressure 29 cm H2O) and an increased dose of acetazolamide. POEMS syndrome was considered as a possible unifying diagnosis. She underwent a skeletal survey which detected a sclerotic bone lesion of the mandible. However, a radionuclide bone scan did not confirm this finding. Despite the lack of a solitary plasmacytoma, she still met the two mandatory criteria (polyneuropathy and M  spike), and the minor criteria of thrombocytosis, papilledema and hemangiomas. The major criterion of elevated VEGF (1799, vascular endothelial growth factor) confirmed the diagnosis of POEMS. Her examination was notable for small hemangiomas distributed along her back and face, diplopia on her right lateral gaze, baseline decreased hearing on the left, distal bilateral lower extremity weakness, decreased sensation to temperature and light touch symmetrically below the knees, and hyporeflexia in her patella and ankles bilaterally. With progressive visual loss, worsening headaches, papilledema, and elevated opening pressures, the patient elected for surgical intervention for a VPS placement with the intent of relieving her elevated ICP. The patient underwent an uncomplicated right frontal VPS placement using MRI registered neurovagination. Her intraoperative manometer ventricular pressure was measured to be elevated at 21 cm H2O. A medium pressure fixed valve was used. She tolerated the procedure without complications and by the evening of postoperative day 1 she noted immediate improvement of her headaches, blurry vision and diplopia. She was discharged on postoperative day 3. Within a month of VPS placement, her headaches, blurry vision, and diplopia had significantly improved, although her vision had not returned completely to baseline. She continued to experience profound fatigue and worsening neuropathy. Rather than local therapy targeted against the source of the disease, she was started on a mycophenolate mofetil taper, transitioned to lenalidomide and dexamethasone, and underwent the first line treatment of autologous peripheral blood stem cell transplantation which has been associated with neurological recovery. The outcome was unknown (recovering from headaches, blurry vision, and diplopia).