Guillain-Barre syndrome - 1 File Download



Guillain-Barre syndromeSymptoms of GBSSymptoms often start in your feet and hands before spreading to your arms and legs.At first you may have:numbnesspins and needlesmuscle weaknesspainproblems with balance and co-ordinationThese symptoms may continue to get worse over the next few days or weeks before they start to?slowly improve. In severe cases, you may have difficulty moving, walking, breathing and/or swallowing.Question: 34 year old lady presented with difficulty walking since last few days. History, examination and management.You are the FY 2 in GP clinic.Dr: Hello Are you Mrs... I am Dr ... How can I help you ?Pt: I am having weakness and numbness in my legs and hands and I am not able to walk properly.Dr: I see. Since when you started having these symptoms ( weakness spreads quickly that within days or weeks in GBS compared to other neurological problems which can months to progress) ? Last few days.Dr: Do you how did these symptoms started ? Pt:These numbness started in my feet and hands and now they are spreading up in the last few days.When do you get these symptoms – any particular time of the day or are they present throughout ?Dr: Did you have these symptoms all these days since it started or are there any days you did not have symptoms ( Multiple sclerosis – sometimes they do not have symptoms) ? I had this every day.Dr: do you have these symptoms in both the legs and both hands or only one side hand and leg ( GBS is bilateral) ? Both the arms and both legs.Dr: Are the weakness is more severe in the evening ( Myasthenia) ? No Dr: Do you have weakness anywhere else – like arms, face, neck ? NoDr: Do you have any other symptoms ? Like what ?Dr: Do you have any pains in arms, legs, back or anywhere in the body (GBS, vasculitis, polymyositis) ? I have pain in my back. Where exactly in your back ? ... Since when ? Since last few days.Dr: Do you have fever ( vasculitis) ? NoDr: Do you feel hot and cold sensations in your legs ( no sensory loss in BGS, myasthenia and polymyositis where as there is sensory loss seen in transverse myelitis,? Yes[ ask symptoms from head to toe]Dr: Any problem in your vision ( Multiple sclerosis, Mysthenia) ? NoDr; Do you have any breathing difficulty ? NoDr: Do you have any problem in speaking ? NoDr: Do you have any problem in swallowing ? NoDr: Do you have diarrheoa or constipation ? NoDr: Do you have bowel or urine incontinence { BGS, Transverse myelitis ( seen early)}? NoDr: Do you have any problem in balance or difficulty walking ( GBS) ? NoDr: any changes in your food recently lie did you have food in restaurants or did you have any canned food recently ( botulism) ? No[ ask triggers for GBS – recent flu or bowel infections]Dr: Did you have fever in the recent past ? Yes, I had flu three weeks ago.Dr: Did you have diarrhoea recently ? NoDr: Did you have this type of problem previously ? NoDr: Do you have any medical conditions or have been diagnosed with medical conditions in the past ? NoDr: Are you taking any medications ? NoDr: Are you allergic to any medications? NoDr: Any family members have any medical conditions ? NoDr: Thank you very much for all the information. Is there anything else you think may be important for us to know ? I don’t think so.Examination:Check the NEWS chart for any temperature.Dr: Mrs I need to examine you now. I need to do what we call as neurological examination.Examiner may give the signs:Power reduced in legs. ( Power was 3) Reflexes will be reduced or absent in GBS, where as in Myasthenia and Botulism they will be normal and hyperreflexia in Tranverse myelitis).Pupil Normal size ( not dilated), Pupils reacts normally to light – in GBS ( Ptosis, dilated and non reactive pupils seen in Botulism)Dr: Mrs.. I could see some weakness in your legs. Investigations :We need to do some tests to find out what exactly is causing these problems.We will refer you to the specialist called Neurologist in the hospital.We need to do tests like Lumbar puncture ( where need to take some fluid from the lower spine and test it)[Elevated cerebrospinal fluid protein without elevated cell count. This may take up to 10 days from onset of symptoms to develop].Also other tests what we call as Eletromyography and nerve conduction test which tests muscle and nerve function. (Abnormal nerve conduction velocity findings, such as slow signal conduction)Examiner may or may not give results. Check for elevated Protein in CSF if CSF result is given.Diagnosis:Dr: Mrs .... I think you have a condition what we call as Guillain Barre syndrome.Do you have any idea about this ? NoDr: Guillain-Barré syndrome is a very rare and serious condition that affects the nerves. It is thought to be caused by a problem with the immune system, the body's natural defence against illness and infection. Normally the immune system attacks any germs that get into the body. But in people with?Guillain-Barré syndrome, something goes wrong and it mistakenly attacks and damages?the coverings of the nerves and reduces nerve function ( condutcting signals from brain to the muscles). This causes weakness in the muscles.Do you follow me ? Yes but how did I get this ?Dr: We do not know what exactly causes this problems. However, we think it is due to previous infection like flu or diarrhoea. In your case you had flu recently. That could have caused this problem.Pt: Is there any treatment doctor?Dr: We need to admit you to the hospital for the treatment. Neurologist will see you and tell you about the treatment.We will a medicine called Immunoglobulin through your veins – Immunoglobulin is made from donated blood that helps bring your immune system under control.We may need to do a procedure called plasma exchange (plasmapheresis)?– an alternative to immunoglobulin where a machine is used to filter your blood to remove?the harmful substances that are attacking your nerves. Our Consultant will decide what is suitable to you. Other treatment we may give are to reduce symptoms and support body functions, such as painkillers.Most people need to stay in hospital for a few weeks to a few months.Do you follow me ? Is that Okay ? Is there anything else you want to know ?Pt: Will I improve after the treatment doctor?Dr: Most people with?Guillain-Barré syndrome make a?full recovery, but this can take months or even years.Some people won't make a full recovery and are left with long-term problems such as:being unable to walk without assistanceweakness in your arms, legs or face, breathing or swallowing problem,numbness, pain or a tingling or burning sensationbalance and co-ordination problemsextreme tirednessTherapies such as?physiotherapy,?occupational therapy?and speech and language therapy can help you recover and cope with any lasting difficulties.Also we may need to put on machine to help with breathing and/or a feeding tube if it is required if there is problem with breathing or swallowing problem in the future.Pt: Will I die because of this problem ? Dr: Most of the people recover from the condition completely. Very rarely only it is life threatening. Any other question ?Warning signs:Dr: In the future after discharge from the hospital if you develop symptoms like difficulty breathing, swallowing or speakingcan't move their?limbs or facefaints?and doesn't regain consciousness within two minutesThis is a medical emergency and you need to be seen in hospital as soon as possibleSo please come to the? A&E department?immediately. Thank you. Differential Diagnosis for GBSDisease/ConditionDifferentiating Signs/SymptomsDifferentiating TestsTransverse myelitisSpinal cord disorders including transverse myelitis present with asymmetric motor or sensory loss usually involving lower extremities, early bowel or bladder dysfunction with persistent incontinence, and segmental radicular pain.Physical exam demonstrates upper motor neuron signs (hyperreflexia, positive Babinski response) and a sensory level.Cerebrospinal fluid (CSF) analysis: pleocytosis with modest number of lymphocytes and increase in total protein.Magnetic resonance imaging (MRI) shows focal demyelination with possible enhancement at the appropriate level. HYPERLINK "javascript:;" \o "Myasthenia gravis" Myasthenia gravisEarly involvement of muscle groups including extraocular, levator, pharyngeal jaw, neck, and respiratory muscles. Sometimes presents without limb weakness.Excessive fatigability and variation of symptoms and signs through the day is common.Reflexes are preserved, and sensory features, dysautonomia, and bladder dysfunction are absent.Electrophysiological study shows normal nerve conduction and presence of decremental response to repetitive nerve stimulation.Electromyogram (EMG) shows abnormal jitter and blocking.Edrophonium test is normally positive.?However, many centers do not routinely perform this test because of potential side effects.Lambert-Eaton myasthenic syndrome (LEMS)Can be difficult to differentiate because of similar clinical characteristics. However, some characteristics are more typical for LEMS. These include slower development of clinical symptoms, dry mouth, lack of objective sensory loss, rare involvement of respiratory muscle group, and potentiation of reflexes after exercise or contraction.? Electrophysiologic study: hallmark is a low amplitude compound muscle action potential (CMAP) after single nerve stimulus, increase in CMAP amplitude after voluntary contraction, or repetitive stimulation at high frequencies.?[137]BotulismHistory of ingesting food tainted with botulinum toxin.Descending paralysis begins in the bulbar muscles then the limbs, face, neck, and respiratory muscles.Respiratory muscles are involved with mild limb weakness, and reflexes are usually preserved.Ptosis, dilated nonreactive pupils are present. Dilated nonreactive pupils are uncommon in GBS, but more common in botulism.Constipation is also a characteristic feature of botulism. Electrophysiologic study: reduced amplitude of evoked muscle potentials, increase in amplitude with repetitive nerve stimulation and increased number of myopathic units, which is atypical for GBS.? PolymyositisPresence of pain and muscle tenderness usually in the shoulder and upper arm, involvement of flexor neck muscle disproportionate to limb weakness, absence of sensory symptoms, preservation of reflexes, absence of dysautonomia, and presence of skin lesions, which are uncommon presentation for GBS. Elevated erythrocyte sedimentation rate (ESR) and creatine kinase (CK), normal nerve conduction study, and myopathic changes with fibrillation on EMG.Muscle biopsy shows muscle fiber destruction and regeneration, and lymphocyte infiltrates.? Vasculitic neuropathyCommon features include painful asymmetric presentation of muscle weakness, uncommon involvement of cranial nerves, respiratory paralysis, and sphincter dysfunction.Usually patients complain of fever, fatigue, weakness, and arthralgia.?[137]May have elevated ESR.CSF does not show albuminocytologic dissociation.Electrophysiologic study shows evidence of denervation.Nerve biopsy shows signs of inflammation and scarring.?[137] ................
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