Anti-Hu syndrome: a rare presentation and a very difficult decision
- Ursula Schulz, Specialist Registrar in Neurology1,
- Bryce Randalls, Consultant Anaesthetist in Intensive Care2,
- Carl Counsell, Clinical Senior Lecturer in Neurology1
- 1Department of Neurology, Aberdeen Royal Infirmary, NHS Grampian University Hospitals NHS Trust, Aberdeen, UK
- 2Department of Anaesthetics, Aberdeen Royal Infirmary, NHS Grampian University Hospitals NHS Trust, Aberdeen, UK
- Correspondence to: Dr U Schulz Department of Neurology, Aberdeen Royal Infirmary, Foresterhill, Aberdeen AB25 2ZN, UK; ursula.schulz{at}nhs.net
Anti-Hu syndrome is the most commonly recognised paraneoplastic condition, typically characterised by a sensory neuropathy or a cerebellar syndrome.1,2 We have recently looked after a patient who—unusually—presented with a rapidly progressive motor syndrome, who was both a diagnostic and an ethical challenge.
HISTORY AND EXAMINATION
A 64-year-old lady presented with a three-week history of painless increasing arm weakness, breathlessness and difficulty walking. By the time she was admitted to hospital her symptoms had deteriorated to the extent that she was unable to lift her arms to feed or to dress herself. She had also started to wear a soft collar because she was unable to hold her head up. Her illness had been preceded by a one-week prodrome of general fatigue and malaise. She had previously been well with no significant past history. She was a longstanding smoker of 50 pack-years.
On examination she looked unwell and was breathless at rest with paradoxical abdominal movements on respiration. Cranial nerve examination showed a slightly weak smile, marked weakness of neck flexion and extension, and a weak cough. There was no muscle wasting or fasciculations. Her deltoids and biceps were very weak bilaterally but distal upper limb power was normal, and there was very mild bilateral weakness of hip flexion. Biceps and triceps jerks were absent bilaterally but other tendon reflexes were easily elicitable. Plantar responses were flexor. There was no sensory deficit and no cerebellar dysfunction. The only abnormality on general examination was a small right pleural effusion. Because of her compromised respiratory function the patient was admitted to the intensive care unit (ITU), where within a few hours her condition deteriorated further and she required intubation and ventilation.
DIFFERENTIAL DIAGNOSES AND INITIAL INVESTIGATIONS
We were presented with a patient with a short non-specific prodrome and then a rapidly progressive lower motor neuron syndrome predominantly affecting proximal …
