A 32-year-old gentleman presented with a history of breathlessness. He had a past history of bronchiectasis. Examination revealed left sided coarse creptitation. He was cyanosed with a pulse rate of 130/m. Blood gas showed PO2 of 56 mm of Hg and PCO2 of 68 mm of Hg, pH-7.2. After a brief trial of non invasive ventilation, he was intubated and started on mechanical ventilation. He was also on broad spectrum antibiotics and respiratory adjuvant therapy. Next day, as his condition was stable, after a successful trial of T-piece for 1 hour, he was extubated. During the visiting hours in the evening, while chatting with his relatives, he suddenly became severely breathless and cyanosed and was reintubated and ventilated. Following extubation after 24 hours, he had another episode of respiratory failure needing reintubation and ventilation.

Belle Vue Clinic Previously Dr Moitra was consultant and In-charge Critical Care Medicine of the Woodlands Hospital, Kolkata. Presently he is the chairman of the Indian Society of Critical Care Medicine, West Bengal Chapter. e-mail: [email protected] Phone: 9831072162 (Emergency Phone), 92370721825, Belle Vue Clinic B.P. Podder Hospital & Medical Research Ltd Phone: 24458901 (1o Lines)

His blood for T3 T4 TSH and CPK were checked urgently which were found to be within normal range. On the 5th day, on careful examination, he was found to have weakness of his upper and lower extremities. On the 6th day, he also developed bilateral facial weakness. A neurological consultation was made and on the 7th day a lumber puncture revealed raised CSF protein (102 mg%) with normal cell count. MRI brain and cervical spine were normal and NCV study was consistent with acute demyelinating polyneuropathy.
Diagnosis
Acute inflammatory demyelinating poly-neuropathy (G. B. Syndrome)
He received 5 days course of IV gammaglobulin. In the course, he developed bulbar palsy and needed percutaneous tracheostomy. After 6 weeks stay in the hospital he was discharged. During discharge, he was still bed bound although he had started moving his extremities with the help of physiotherapy. After 3 months his tracheostomy was withdrawn and he was managing to walk with zimmer frame with the help of a physiotherapist.
[lnterestingly his elder brother was admitted under our care 6 years back with features of bilateral facial palsy followed by quadriparesis. He also received IV gammaglobulin and made a good recovery. Fortunately, he did not need any respiratory assistance with intubation or ventilation. The link between these two cases in the same family could not be established].
Discussion
Although not so common in primary care practice, critical care physicians frequently confront with Guillain-Barre syndrome. Although, easy to detect when it presents with typical features, diagnosis may be delayed if the classical features are absent during initial presentation.
Classical presenting features:
Usually there is an antecedent history of upper respiratory tract infection or diarrroea
Numbness, tingling, pain and weakness
Weakness worse in legs than arms
Usually symmetrical
Reflexes are lost
Objective sensory signs may not be present
Half will have cranial nerve and bulbar nerve involvement
Weakness increases to its maximum 1-2 weeks from onset and 20% will require mechanical ventilation
Pain may be a prominent feature
Autonomic disturbances like sudden changes in BP and heart rate are common.
Sometimes the patient may present with respiratory failure because of respiratory muscle weakness.
Bilateral facial palsy can also be a presenting feature.
Although unilateral facial palsy is easy to diagnose, inexperienced clinician may miss the diagnosis of bilateral facial nerve palsy because of symmetric involvement. Careful examination of eye closure, blowing and whistling test will make it obvious.
Common causes of bilateral facial nerve palsy
1. Gullain Barre Syndrome
2. Sarcoidosis - with bilateral parotid gland enlargement]
3% of Gullain Barre syndrome present with features of opthalmoplegia, areflexia and ataxia (Miller-Fisher syndrome)]. There may not be motor weakness.
Differential diagnosis
In the setting of acute neuromuscular paralysis, the diagnosis of GB syndrome is largely clinical. However, it has to be differentiated from some other conditions like:
Poliomyelitis - Asymmetrical, pure motor, CSF pleocytosis.
Transverse myelitis- Acute, lower extremities, symmetrical predominant sensory findings.
Myasthenia gravis- Pure motor, tests like EMG.
Polymyositis - Muscle pain and raised CPK.
Motor neurone disease- Chronic disease, mixed upper motor neurone and lower motor neurone signs, EMG.
Hypokalaemia- Serum potassium level
Botulism- Typical history
Critical care myopathy- Typically in long stay ITU patients - on steroids, high CPK.
Diagnosis
1. LP shows raised protein with normal cells (may be normal in first 2 weeks).
2. NCV study - shows spectrum of motor and sensory involvement. (18% pure motor involvement). Predominantly there is demyelinating neuropathy although axonal involvement with minimal demyelination can also occur.
3. Pure motor variety is usually associated with Campylobacter and anti gM1 antibodies and severe sensory variety with Cytomegalovirus and anti gM2 antibodies.
Predicting prognosis
Following factors are associated with high probability of poor outcome.
1. Preceding history of diarrhoea.
2. Rapid onset - bed bound within 2 days.
3. Requirement of artificial ventilation.
4. Reduced or inexcitable motor action potential amplitude.
Management
The most important part of the management is to recognize impending respiratory failure and intubate and ventilate in appropriate time. The patient should be managed in ITU and oxygen saturation, respiratory and abdominal muscle movement should be watched carefully (increased abdominal muscle movement is a warning sign). Increased secretion and swallowing problem should also be noticed. Their vital capacity and peakflow should be observed regularly. Any other the suggestive sign should prompt the clinician to proplylactically intubate and ventilate. [Most of the deaths occur due to delayed intubation in the event of a cardiac respiratory arrest with a very poor outcome).
Other aspects of general care
1. Physiotherapy
2. Urinary catheterization    
3. Nutrition
4. Deep vein thrombosis prophylaxis (very important)
5. Monitoring automatic function
Sudden bradycardia and cardiac arrest is a common cause of death. One has to be careful about this while doing suction. Proplylactic atropine and even pacemaker insertion may be needed.
Specific therapy
Two forms of therapy are effective.
1. IV gammaglobulin (0.4 gm/kg IV daily × 5 days) :
It can be useful even after 2 weeks of onset of weakness. Headaches, meningism and eczema of the hands are uncommon side effects. Anaphylaxis is rare.
2. Plasma exchange is equally helpful. However it is more hazardous and inconvenient. Moreover the chance of viral transmission is there. Therefore it is preferable to use IV gammaglobulin in most of the clinical situation.
Our experience
In last 10 years we have treated 63 patients presenting with G.B syndrome.
All of tl1em received IV gammaglobulin. 26 patients made an uneventful recovery. 10 patients needed only intubation for tracheal toileting, 27 patients needed mechanical ventilation, 10 patients died - 3 of them due to sudden cardiac arrest and 5 of them due to sepsis related problems. 5 of the patients had only cranial nerve palsy (bulbar, seventh and ocular) one of them died because of aspiration pneumonia.
A 56-year-old lady presented with swallowing problem with choking sensation. She had a loss of gag reflex. Initially there was a diagnostic dilemma. Later on during her stay she developed bilateral seventh nerve palsy as well as opthalmoplegia.
She eventually needed tracheostomy. L. P. and NCV studies were non-diagnostic. Although she received IV gammaglobulin, it did not shorten the course of her illness. She was in ITV for nearly six weeks. At present she is off tracheostomy tube.
A 29-year-old lady presented with diplopia. Clinical examination revealed opthalmoplegia due to bilateral 3rd and 6th nerve palsy. She also developed right sided 7th nerve palsy. MRI brain was normal. Lumber puncture and NCV studies were non-contributory. With a provisional diagnosis of a typical G.B syndrome, she was given IV gammaglobulin. She made a complete recovery within next 10 days.
A 62-year-old gentleman was admitted with profound weakness of all extremities. He gave a history of diarrhoea for 3 days before admission. Examination revealed quadriparesis without any bladder involvement. Clinical diagnosis of G.B syndrome was made. Blood reports showed 'K' of 2.1 mg%. He was started on 'K' replacement and IV gamtnaglobulin. The weakness started improving within 24 hours and his 'K' level became normal within 48 hours. He also completed the course of IV gammaglobulin for 5 days.
He underwent lumber puncture which was normal. He also underwent NCV study which was also normal. Before discharge, he was also given glupose challenge (to exclude hypokalaemic periodic paralysis) without any subsequent fall of serum 'K' level. He was discharged on the 6th day with absolutely normal muscle power.
Diagnosis
Diarrhoea induced severe hypokalaemic paralysis simulating G.B syndrome.
A 42-year-old gentleman was admitted from the clinic for treatment of sero positive rheumatoid arthritis with severe pain in the joints. On the first day he received methyl Prednisolone pulsing (1gm) and he felt much better as far as the pain was concerned. However, he complained some weakness in the body. Following the 2nd dose next day, his pain improved further but during the evening round he distinctly complained of weakness of his upper extremities.
At night the nurse had to rescue him from the toilet as he fell and could not lift himself up. A detailed clinical examination revealed weakness of upper and lower limbs (power grade 1/5). Blood tests for CPK, electrolyte, TSH were sent urgently and his 'K' was found to be 1.9 mg%.
He was given urgent 'K' replacement and methyl prednisolone was not given any further. Within next 48 hours he regained his muscle power and his 'K' level rose to normal limits. Although initially scared and grumbling (as he thought that his paralysis was iatrogenic) he left the hospital happy and contended.
Diagnosis
Pulsed methyl prednisolone induced severe hypokalaemia with quadriparesis.
[Pulsed methyl prednisolone can also give rise to hypertensive crisis, acute gastric perforation and severe hypokalaemia. The patients should be monitoring closely during therapy].
Diagnosis
A 40-year old lady presented with acute generalized weakness of all extremities. Examination revealed muscle power of O/5. All the deep reflexes were brisk and the cranial nerves were well preserved. Her LP and NCV tests were normal. Because of inconsistencies in clinical findings, she was referred to a psychiatrist and later a diagnosis of hysterical conversion reaction was made.
A 30-year-old lady (wife of a doctor) presented with weakness of extremities. Examination: Motor and sensory - normal, ankle jerks lost, walked with ataxic gait, L.P - non contributory. Initially it was thought to be functional. Later on she developed bilateral ptosis, NCV showed evidence of demyelination. She was diagnosed to have Miller-Fisher syndrome. She responded well to I.V. gamma globulin.
A 56-year-old gentleman who was admitted with fracture neck, femur undergoing surgical fixation developed post operative deep vein thrombosis of right leg needing a medical consultation from our team. He was treated appropriately and before discharge he was c01f1plaining of tingling and numbness few more than 3 months of upper and lower extremities which led to his fall.
Neurological opinion was taken and detailed neurological examinations done, which showed loss of deep reflexes indicating evidences of peripheral neuropathy. Following this opinion, lumbar puncture was done which showed increased protein with normal cell count. Nerve conduction studies done later on showed chronic inflammatory demyelinating polrneuropathy (CIDP).
He was treated with in steroids, followed by oral steroids for 3 months with substantial improvement of symptoms.
Diagnosis
Chronic inflammatory demyetinating polyneuropathy (CIDP).
2 Years later he presented with sudden onset of weakness of both legs while he was returning from his holiday. Examination revealed weakness of both legs. Power O/5 of lower limbs and 2/5 of upper extremities with loss of reflexes.
Lumbar puncture showed increased protein with normal cell court and nerve conduction study was consistent with CIDP.
Diagnosis : relapsing CIDP
This time after neuro consultation he was treated with iv gammaglobulin, which showed dramatic improvement of muscle power within a week's time. Following discharge he received monthly iv gammaglobulin 400 mg/kg one dose every month for 6 months.
At present he is doing well.
Discussion
CIDP can sometimes be confused with GB syndrome but symptoms is usually present for 3 months duration as opposed to GB syndrome, which is of acute onset.
Treatment
Treatment of CIDP is with steroid and immunoglobilin with variable result.
It can take a relapsing course. Once diagnosed, it should be treated with the help of a neurologist.
Key Points
G.B syndrome is an important cause of acute neuromuscular paralysis.
It should be differentiated from other causes like poliomyelitis, transverse myelitis, myasthenia, hypokalaemia and cerebrovascular accidenty by clinical, laboratory tests and neuropysiological studies. IV gammaglobulin is the treatment of choice.
Impending respiratory muscle failure should be anticipated early by clinical and other parameters (like vital capacity; peak expiratory flow etc.)
Autonomic failure is common which can lead to sudden cardiac arrest. Care should be taken during suction and proplylactic pacemaker may be needed.
IV gammaglobulin can be given even upto 2 weeks of onset of paralysis.
Prophylaxis for DVT is very important in paraplegic patients, as there may be sudden death due to pulmonary embolism.(Reprint)
Source:  “Experience with Evidence in Clinical Practice,”
Dr. Subrata Maitra