Age: 41.0
Sex: F
Vaccine: FLUX, FLUX
Symptoms: Areflexia, Bone scan normal, CSF pressure increased, CSF protein increased, CSF shunt operation
Clinical narrative: Case number PHHY2015US071984 is an initial literature report received on 13 Jun 2015. The author presented the report of a patient with POEMS syndrome with papilledema and elevated intracranial pressure (ICP) refractory to medical management requiring ventriculoperitoneal shunting (VPS). This report refers to a 41 year old female patient who was healthy. She was vaccinated with influenza vaccine (manufacturer and batch number: not reported) on an unknown date. Within one week of influenza vaccination, she developed bilateral numbness and paresthesia in her hands and feet. Her examination was notable for distal lower extremity weakness, 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 (acute onset of sensory-motor polyneuropathy with prolonged F wave latencies, showed 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 medical gastrocnemius). She was diagnosed with Guillain Barre syndrome and treated with multiple rounds of intravenous immunoglobulin followed by CELLCEPT. 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 with a value of 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. With consideration of her clinical course, these findings were thought to be most consistent with chronic inflammatory demyelinating polyneuropathy (CIDP). Given her worsening polyneuropathy, she underwent a magnetic resonance imaging (MRI) of the spinal cord which demonstrated a 2.1 x 1.4 x 3.6 cm homogeneously enhancing mass in the right T12/L1 neural foramen with expansion into the right epidural space. She underwent a T12 laminectomy for resection 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 computed tomography (CT) scans, MRI, 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. Given a concern that her clinical picture was not entirely consistent with CIDP, POEMS syndrome was considered as a possibly 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 author stated that in this patient, the nerve conduction studies and electromyography demonstrated acquired demyelinating polyneuropathy and axonal loss which in conjunction with her other exam and laboratory findings, were consistent with POEMS diagnosis. The major criterion of elevated vascular endothelial growth factor (VEGF) with a value of 1799 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 with symptoms of hydrocephalus, 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 neuronavigation. Her intraoperative manometer ventricular pressure was measure to be elevated at 21 cm H2O. A median 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 was currently undergoing first line treatment of autologous peripheral blood stem cell transplantation which was associated with neurological recovery. The author stated that the decision to pursue VPS placement arrived only after exhausting the alternative therapeutic options. The presence of shunt in an immunosuppressed state (as POEMS syndrome treatment involved multiple rounds of immunosuppressive therapy including autologous stem cell transplant) raised a significant concern for shunt complications including infection, malfunction and wound break down. However, given her rapid visual decline, the decision was made collectively that VPS placement was the appropriate course of action to preserve her vision and mitigate her symptoms of elevated ICP.