• amyloid neuropathy
• lymphoma

We report a 70 year old German man presenting with a three year history of progressive numbness and painful tingling and burning paraesthesiae in his hands, feet, and lower legs, which had worsened during recent months. He also reported unsteadiness of gait, fatigue, night sweats, loss of appetite, and a weight loss of 12 kg within one year. He denied bowel or bladder problems and alcohol ingestion, but admitted smoking (110 pack-years). His family history was negative for neurological diseases.

The family was originally from Gdansk (now Northern Poland). The patient's father and his four siblings all reached their 80s without developing neurological symptoms. The patient's mother died at the age of 64 of blood cancer, and her half brother died at the age of 78. Three of the patient's siblings died at the ages of 1, 17, and 33 (starvation, killed in the war, stomach cancer). Two further sisters, aged 64 and 69, their descendants, and the patient's own five sons and their children were healthy. Both of the patient's grandmothers died in their 80s, whereas the paternal grandfather died early of unknown cause, and the maternal grandfather drowned in his 30s.

Neurological examination revealed severe ataxia of gait and stance, atrophy of the small hand and foot muscles, and bilateral distal pareses (3–4/5 on the MRC scale), diminished tendon jerks, a glove and stocking distribution of hypoaesthesia for all sensory …