Poster Abstracts / JAMDA 15 (2014) B3eB28B4

Back Pain, Tingling and Fatigue. Is It Early Guillain-Barr Syndrome?

Presenting Author: Mazen Matar, Hull and East Yorkshire Hospitals NHSTrustAuthor(s): Mohamed Farag; and Mazen Matar

Introduction: Acute polyneuropathies present a diagnostic challenge inmedical practice. A 51-year-old female presented with 3-day history ofmild back pain, mild right facial tingling, distal limb paraesthesia and fa-tigue. Urgent head MRI was normal, and spine MRI showed mild-to-moderate cervical disc herniation at C5/C6 without neural impingement.Because full neurological examination was normal and symptoms werenot explained by the prolapsed disc, shewas dischargedwith a diagnosis ofprobable anxiety-related paraesthesia. Three days later, due to aworseningof her symptoms and increased urinary frequency, she was readmitted.Further deterioration, including progressive facial diplegia and quad-riparesis, was observed. Physical examination, lumbar puncture and nerveconduction studies were consistent with Guillain-Barré syndrome, likelypost-viral. Intravenous immunoglobulins and physiotherapy led toconsiderable improvement of her symptoms. In the early stages of acutepolyneuropathy, symptoms might be non-specific, and the results ofroutine investigations might be misleading.Case Description: A 51-year-old, previously healthy, Caucasian femalepresented to the medical assessment unit of a general district hospitalwith a 3-day history of mild aching pain in the back, tingling in the hands,feet and right side of the face and fatigue. There was no significant pastmedical history and she was not on any regular medications. The patientwas an ex-smoker and, until 5 years ago, drank moderate amounts ofalcohol. A full neurological assessment was unremarkable and wascharacterized as follows: normal cognition, normal cranial nerve exami-nation including fundi, intact sensation in all modalities (light touch, pain,vibration, joint position and temperature), no muscle wasting or fascic-ulations, normal muscle tone and normal power in all four limbs. Tendonreflexes were normal and symmetrical, including ankle jerks, which wereelicited bilaterally. Clonus in the limbs was negative, and plantar responsewas down-going bilaterally. There were no signs of ataxia or dysmetria inthe upper or lower limbs, with normal gait and a negative Romberg sign.Routine blood investigations were within normal ranges. Although theneurological examination was normal, neuroimaging was prescribed inlight of the sudden onset of subjective symptoms. An urgent MRI scan ofthe head was normal. A cervical spine MRI showed a mild-to-moderatecentral subligamentary disc bulging at C5/C6, indenting the anteriorthecal sac and cord in the caudal direction, with an element of unco-vertebral arthrosis. The exiting nerve roots appeared intact. Upon furtherquestioning, the patient admitted having long-standing intermittent mildneck pain without radiation to the upper limbs. She denied any history ofhead or neck trauma. The neurosurgical team decided that her presen-tation was not related to the herniated cervical disc, given its location.Although ipsilateral facial sensory disturbances may occur with highcervical lesions, her spinal cord and nerve roots did not show any changesin the neuroimaging. Furthermore, paraesthesia in the feet could notimmediately be attributed to the herniated disc. Subsequently, the patientwas diagnosed with probable anxiety-related paraesthesia and dis-charged with an outpatient review scheduled for the following week.Three days later, the patient presented to the Emergency Departmentwith evolving symptoms: increased urinary frequency, unsteadiness onher feet and temperature sensory disturbance in all limbs. The tinglingsensations were worse in all limbs and on both sides of her face. Addi-tionally, she presented with occipital headaches and a severe aching-typepain in the region of the thoracic spine. Further history revealed flu-likeillness 3 weeks before the initial presentation. The patient denied anyhistory of skin rash, foreign travel, or exposure to toxic materials or tickbites. Within the first 24 hours of hospital stay, she developed progressivebilateral facial palsy. Neurological examination revealed bilateral, com-plete lower motor neuron facial palsy and decreased power (grade 4 outof 5) in all limbs. Triceps, supinator, and ankle deep tendon reflexes wereabsent bilaterally, but bicep reflexes were preserved. Sensory examina-tion revealed symmetric loss of the vibration sense in the lower limbsfrom the feet up to both knees and a positive Romberg sign. The rest of

the neurological examination was unremarkable: normal cognitivefunctions, intact other cranial nerves, normal muscle tone in all fourlimbs, down-going plantar reflexes, absent clonus, intact sensation totouch and pain and normal coordination were observed. No breathingdifficulty was experienced by the patient during the illness. Lumbarpuncture showed clear acellular cerebrospinal fluid (CSF), high proteinlevels (1.9 g/l), normal glucose levels and negative Gram film. A presumeddiagnosis of Guillain-Barré syndrome was made. Other investigationsincluded CRP, FBC, U&Es, LFTs, thyroid profile, coagulation profile, andhaematinic screen.Discussion: Polyneuropathy, or simultaneous malfunction of various pe-ripheral nerves throughout the body, can be either acute or chronic. Pol-yneuropathies usually develop gradually over days, months or even years.Acute polyneuropathy has many different causes, and with a detailedhistory, full neurological examination, blood investigations, neurophysio-logical assessment and neuroimaging, it usually has an identifiable un-derlying cause, though this can be challenging to identify. Focal discprotrusion or herniation is frequently found on anMRI of the cervical spinein asymptomatic patients. In an American study, the incidence of cervicalfocal disc protrusions in asymptomatic volunteers was 50%. (Ernst, Eur JRadiol, 2005) In symptomatic patients, however, their presence cansometimes be confounding, especially if multiple conditions that causesimilar symptoms are present. Bilateral facial nerve paralysis is rarely seenin medical practice, and it can be caused by traumatic, infectious, neuro-logical, metabolic, toxic, vascular, or idiopathic conditions. The mostcommon infectious cause of facial diplegia is Lyme disease. Our patientwas not checked for Lyme disease, as she had no history of rash, travelabroad or tick bites. Normal MRI of the head excluded multiple sclerosisand rare conditions such as CNS leukemia, lymphoma, and meningealcarcinomatosis. In view of normal blood investigations, including CRP,calcium, autoimmune screen and ACE levels, other systemic illnesses, suchas sarcoidosis, infectious mononucleosis and vasculitis conditions (such assystemic lupus erythrematosus and polyarteritis nodosa), were consideredless probable. The patient’s history and presentation were not suggestiveof herpes viral infection as a cause of facial diplegia; therefore, screeningfor herpes viruses (herpes simplex and varicella-zoster viruses) was notindicated. Guillain-Barré syndrome is a rare, acute inflammatory demye-linating polyneuropathy (AIDP). GBS is usually a post-infectious immune-mediated disease. Infectious agents induce the production of antibodies,which then cross-react with specific gangliosides and glycolipids that aredistributed throughout the myelin in the peripheral nervous system (PNS).Typically, GBS is characterized by rapidly progressive, symmetricallyascending muscular weakness and areflexia. However, it may cause sen-sory disturbance, pain and dysfunction of the autonomic nervous system.Our patient’s presentation was considered to be typical GBS. Steroidtreatment of GBS is ineffective, unlike more chronic demyelinating neu-ropathies, which may respond well to steroids. Plasmapheresis andintravenous immunoglobulins have been proven to be equally effectivetreatment options and can hasten recovery. Respiratory function should beclosely monitored due to the possibility of respiratory failure secondary todiaphragm muscle paralysis. There is robust evidence supporting theimportant role of physiotherapy and occupational therapy in regainingmuscle strength, normal gait and functional independence. Prognosis incases of AIDP is generally good, with most patients recovering completelywithin a few months. However, recovery may be slow and not alwayscomplete if the patient suffers from acute motor and sensory axonalneuropathy (AMSAN), which is considered to be a severe variant of GBS.This patient’s presentationwas rather challenging and complex initially, asthe presence of an asymptomatic cervical disc prolapse was initially aconfounding factor in the evaluation of her rare medical condition. Thishas resulted in delay in the diagnosis and management, with more pa-tient’s distress. A low threshold strategy for suspecting GBS should beadopted in patients presenting with unexplained neurological signs orsymptoms after recent infections. In complex neurological presentations,general physicians should seek a neurologist’s opinion early to minimizemisdiagnoses.Author Disclosures: All authors have stated there are no financial dis-closures to be made that are pertinent to this abstract.