Age: 41.0
Sex: F
Vaccine: FLU3
Symptoms: CSF protein increased, Computerised tomogram normal, Demyelinating polyneuropathy, Diplopia, Electrophoresis protein abnormal
Clinical narrative: This case was reported in a literature article and described the occurrence of poems syndrome in a 41-year-old female patient who received Influenza vaccine. On an unknown date, the patient received Influenza vaccine at an unknown dose. On an unknown date, 7 days after receiving Influenza vaccine, the patient experienced poems syndrome (serious criteria GSK medically significant), demyelinating polyneuropathy (serious criteria GSK medically significant), Guillain Barre Syndrome (serious criteria GSK medically significant), papilledema (serious criteria hospitalization and GSK medically significant), hydrocephalus (serious criteria GSK medically significant), numbness, paresthesia, sensory loss, spinal hemangioma, frontal headache, blurred vision, diplopia, pulsatile tinnitus, visual acuity decreased, hearing decreased, monoclonal gammopathy and thrombocytosis. The patient was treated with prednisone, acetazolamide, immunoglobulins nos, CELLCEPT, mycophenolate mofetil, lenalidomide and dexamethasone. On an unknown date, the outcome of the poems syndrome, demyelinating polyneuropathy, guillain barre syndrome, numbness, paresthesia, sensory loss, pulsatile tinnitus, hearing decreased, monoclonal gammopathy and thrombocytosis were unknown and the outcome of the papilledema, frontal headache, blurred vision, diplopia and visual acuity decreased were recovering/resolving and the outcome of the hydrocephalus was recovered/resolved and the outcome of the spinal hemangioma was not recovered/not resolved. It was unknown if the reporter considered the poems syndrome, demyelinating polyneuropathy, guillain barre syndrome, papilledema, hydrocephalus, numbness, paresthesia, sensory loss, spinal hemangioma, frontal headache, blurred vision, diplopia, pulsatile tinnitus, visual acuity decreased, hearing decreased, monoclonal gammopathy and thrombocytosis to be related to Influenza vaccine. Additional information was provided: This case was reported in a literature article and it described the occurrence of POEMS syndrome in a 41-year-old female patient who had received an unspecified influenza vaccine (manufacturer unknown). The author reported the patient was otherwise healthy. No further information on the patient's concurrent medical conditions, medical or family history, or concomitant medication was provided. On an unspecified date, the patient received an unspecified influenza vaccine (dosage unknown, administration route and site unspecified; batch number not provided). On an unspecified date, 1 week after the vaccination, 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 but an otherwise normal cerebrospinal fluid profile. Nerve conduction studies demonstrated features that were suggestive of an acquired demyelinating polyneuropathy and axonal loss. The patient was diagnosed with Guillain Barre syndrome and treated. Despite treatment, she had persistent lower extremity weakness along with sensory loss in her bilateral lower extremities and hands. A repeat cerebrospinal fluid examination was notable for protein. A serum protein electrophoresis study demonstrated elevated free lambda light chain with an abnormal banding pattern. Subsequent immunofixation confirmed monoclonal gammopathy with a dual immunoglobulin A M-spike and two immunoglobuline A lambda M components of 0.15g/dl and 0.06g/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. Given her worsening polyneuropathy, she underwent a magnetic resonance imaging of the spinal cord which demonstrated a 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. However the patient did not experience resolution of her symptoms following surgery. Approximately 6 months after the initial onset of symptoms, the patient 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 scans, 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 and 20/30 oculus sinister. Prednisone dose was increased and she was started on acetazolamide. She was subsequently admitted to the neurology service for working of her persistent headaches, progressive visual deterioration and papilledema. Over 2 weeks, her visual acuity decreased to 20/70 oculus dexter and 20/60 oculus sinister, 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 chronic inflammatory demyelinating polyneuropathy. POEMS syndrome was considered as a possible unifying diagnosis. The patient underwent a skeletal survey which detected a sclerotic bone lesion of the mandible. However, a radionuclide bone scan did not confirm this finding. According to the authors, 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 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 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 ventriculoperitoneal shunting placement with the intent of relieving her elevated intracranial pressure. This was a serious case as the patient required hospitalisation. On an unspecified date a lumbar puncture demonstrated a modestly elevated protein level of 0.55 g/L (normal range no provided) level but an otherwise normal cerebrospinal fluid profile. Furthermore 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 tibials anterior and medical gastroenemius). A repeat cerebrospinal fluid examination was notable for protein of 1.15g/L (normal range no provided). A serum protein electrophoresis study demonstrated elevated free lambda light chain (31; normal range: 5.7-26.3) with an abnormal banding pattern. Given her worsening polyneuropathy, she underwent a magnetic resonance imaging of the spinal cord which demonstrated a 2.1x1.4x3.6 cm homogeneously enhancing mass in the right T12/L1 neural growth factor (1799, normal range: 31-86) which confirmed the diagnosis of POEMS, according to the authors. At the moment the patient was diagnosed with Guillain Barre syndrome she was treated with multiple rounds of intravenous immunoglobulin followed by CELLCEPT (Genenteh). The patient underwent an uncomplicated right frontal ventriculoperitoneal shunting placement using magnetic resonance imaging registered neuronavigation. Her intraoperative manometer ventricular pressure was measured to be elevated at 21 cm H2O. A medium pressure fixed valve (Medtronic Sofamor) 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. The patient was discharged on postoperative day 5. Within a month of ventriculoperitoneal shunting 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, the patient was started on a mycophenolate mofetil taper, transitioned to lenalidomide and dexamethasone and, at the moment of reporting, the patient was undergoing first line treatment of autologous peripheral blood stem cell transplantation which has been associated with neurological recovery. The authors did not comment on an causal relationship between the vaccine and the event. The authors concluded that "we present a 42-year-old woman with a 1 year history of treatment of refractory polyneuropathy, monoclonal gammopathy, thrombocytosis, papilledema, hemangiomas and elevated vascular endothelial growth factor, consistent with the diagnosis of POEMS syndrome. She also demonstrated symptoms of hydrocephalus with elevated lumbar puncture opening pressures and the decision was made to proceed with ventriculoperitoneal shunting placement. to our knowledge, this is the first report of ventriculoperitoneal shunting for hydrocephalus in the setting of POEMS syndrome".