A 60-year-old man reported slowly progressive symptoms over a period of five years. He experienced tingling in his toes that gradually spread halfway up the lower legs. He also developed a stiff feeling in his lower legs and nightly cramps in both calves, but no pain. For one year, he complained of numb fingertips with some loss of dexterity. He had no symptoms of autonomic dysfunction, was not known to have diabetes, ate a healthy, balanced diet, drank one glass of alcohol a day, and had not been treated with neurotoxic medication. His family history indicated no other relatives with similar complaints.

A 49-year-old man noticed a diminished sensation in his feet. Two months later, climbing stairs became difficult due to proximal muscle weakness of his legs. Over the next three months, sensory disturbances and both proximal and distal weakness progressed in arms and legs requiring the use of a walker or a wheelchair. Besides COPD, he was previously in a healthy condition. Based upon ancillary investigations, he was initially diagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP), had received a course of intravenous Ig (IVIg), and was subsequently treated with high-dose corticosteroids (60 mg/day). He did not smoke or use drugs such as nitric oxide.

A 40-year-old man was referred because he wished to be informed about the genetic nature of his disorder. He was diagnosed with Charcot–Marie–Tooth (CMT) disease. At 14 months of age, he started walking, but awkwardly due to a bilateral drop foot for which braces were prescribed. On first examination at age 2 years and 8 months, there was marked atrophy, hypotonia, and areflexia of the lower legs, and slight wasting of the thenar and hypothenar. At that time, nerve conduction studies showed normal motor conduction velocities of arm nerves. No motor unit action potentials could be recorded in the lower leg muscles on concentric needle examination.

A 20-year-old previously healthy man suddenly noticed that he was unable to run. The next day he could not climb the stairs and lost strength in his arms. He was admitted to hospital, and over the next hours he progressively lost muscle power in his arms and legs. Swallowing was progressively impaired, and he noticed minor tingling in both hands and feet. He had had a minor upper respiratory tract infection a week prior to admission.

A 53-year-old man developed a left-sided foot drop and a painful sensation on the ventral side of the foot and outer part of the lower leg. Two weeks later, the same symptoms also developed on the right side. In addition, he noticed progressive numbness of his lower legs. Three weeks later, he noticed weakness of his right hand, and was unable to spread his fingers. He had no other symptoms, and his medical history was not informative. He did not recall a tick bite or erythema migrans, or any pulmonary abnormality, and had not visited tropical countries. He does not sit with crossed legs.

A 66-year-old man with slowly progressive tingling and a dull feeling in his feet for two years visited our outpatient clinic. He noticed some imbalance when walking after rising from a chair or from bed. He had no complaints about his hands, and he did not notice weakness. He loved to play golf with his friends several times a week. He was known to have a steatotic liver and hypertension. He did not smoke, but he admitted that he had been drinking six glasses of beer or wine a day for many years. He used anti-hypertensive drugs and vitamin B complex.

A 76-year-old man complained about progressive dull feelings and weakness of the distal lower limbs that gradually progressed over a couple of months to the proximal legs and the hands. In addition, there was minor myalgia in the proximal muscles. He had had a myocardial infarction with cardiac arrhythmia three years earlier. He was treated with amiodarone afterwards. He did not have visual complaints and was otherwise healthy. He did not drink alcohol or use other drugs. He had not been treated with cytostatic drugs.

A 73-year-old-woman noticed pain in her right lower leg and thigh and left foot. After a few weeks of physiotherapy, she gradually developed tingling in her feet and a ‘plastic’ sensation in the soles of her feet. A few weeks later, her feet became completely numb, and she noticed painful tingling in her hands and around her left knee. Because of the tingling in her hands, she could barely use a fork and knife. Walking became difficult due to the dull feelings in her legs. Several drugs against painful neuropathy did not help. For years she had smoked two packs of cigarettes a week. A total of 50 pack-years was estimated.

A 76-year-old man was referred with a diagnosis of ‘motor neuron disease’ or ‘polyneuropathy’. For about eight months he had noticed progressive muscle weakness in his right leg associated with pain, initially more intense than at referral, and a numb feeling around the right knee. Two years previously his left hand had become weak and wasted, associated with loss of sensation of the ring finger, the little finger, and the ulnar part of the palm of his hand. He had lost weight (5 kg) unintentionally over the past six months. He had always been very active, but was now no longer able to walk his dog or do some gardening. He was recently diagnosed with diabetes mellitus (DM) for which oral antidiabetics were prescribed. Family history was unremarkable.

A 70-year-old man noticed a feeling of walking on cotton wool for the past two years. Numbness had progressed from the toes to the knees, and for half a year there was tingling and numbness in the fingertips. These complaints were symmetric and there was no pain. Walking had become insecure. For one year he had no erections, whereas sexual function had previously been normal. There were no other signs of autonomic dysfunction. In the past two years there was also shortness of breath on exertion. A diagnosis of cardiomyopathy had been made recently.

The family history revealed vitreous opacities in the father and several siblings. A brother also had sensory disturbances in his feet and a thickened heart muscle. His four daughters did not have any complaints.

A previously healthy, very active 68-year-old man, who usually cycled over 100 km several times a week, noticed progressive tingling in his feet and lower legs that increased over several weeks. This was followed by progressive weakness in the arms and legs exceeding a period of eight weeks. After three months of progression, weakness became so severe that he could not even walk without help. He did not use drugs or drink alcohol.

A 62-year-old man reported slowly progressive symptoms over a period of two years. He could no longer walk steadily and developed numb feelings on the soles of both feet and a tremor of both hands. He was not known to have diabetes and ate a healthy, balanced diet, drank one glass of alcohol a day, and had not been treated with neurotoxic medication.

A 24-year-old woman had difficulty with walking since early childhood. At age 18 months she was able to walk without support. She often stumbled and could not keep up with her peers in gym class. However, she still had been able to walk 5 km during a four-day walking event. Management included physiotherapy, and she had orthopaedic shoes. She underwent surgery at age eight years (tendon repositions of both feet). Family history was not available because she was adopted. Previous history includes bilateral congenital hip dysplasia and congenital hypothyroidism.

Guillain Barré syndrome (GBS) is an acquired neuromuscular disorder that presents as rapidly progressive weakness and numbness, usually in an ascending fashion. The underlying pathophysiology is monophasic inflammatory immune-mediated demyelinating polyneuropathy that in many cases is triggered by viral illnesses, usually a few weeks prior to the onset of syndrome. The majority of cases are in young adults, although it can occur in pediatric and elderly age groups. GBS should be considered in the differential diagnosis of new-onset rapidly progressing weakness in an otherwise healthy individual.

Key Learning Points

1. 1. Several conditions exist that may lead to profound muscle weakness and death.

2. 2. The most common conditions leading to admission to the ICU are Guillain–Barré syndrome and myasthenia gravis.

3. 3. Early identification and supportive care can be lifesaving.

4. 4. Specific therapies initiated in a timely manner can improve the prognosis significantly.

5. 5. ICU-acquired weakness should be considered in ICU patients with flaccid, generalised weakness or weaning difficulties.