The familial disease that John Russell Silver first described 40 years ago has spawned research into a wide range of neurological diseases
- By: John Russell Silver
It is impossible to predict which of the thousands of papers published each year will be important and which will be consigned to the dustbin of obscurity. Only time will show their proper role, but the exact way in which a disease was recognised is easily forgotten.
In 1959, at the beginning of my career I was working as a registrar in neurology at the Middlesex Hospital, when a young girl presented with a wasted hand and spastic lower limbs. She denied that any other members of her family were affected. I thought that she had an intrinsic tumour of the spinal cord and she was admitted for investigation, but a myelogram found no abnormality.
She subsequently developed a sterile meningitis and became very ill, and her mother came to visit her in hospital. The house officer shrewdly observed that the mother had a similar spastic gait to that of