Table 2.

Recommended intervals for different types of visits and examinations

Coutinho stage	0	1	2	3	After change in medication
Patient history (recommended intervals in months)
Sensorimotor symptoms	(6–) 12	6 (–12)	(3–) 6	(3–) 12	3
Autonomic symptoms	(6–) 12	6 (–12)	(3–) 6	(3–) 12	3
Medication	(6–) 12	6 (–12)	(3–) 6	(3–) 12	3
Neurological examination (recommended intervals in months)
Qualitative sensory status	(6–) 12	6 (–12)	(3–) 6	(6–) 12	3
Distal muscle strength	(6–) 12	6 (–12)	(3–) 6	(6–) 12	3
Gait stability	(6–) 12	6 (–12)	(3–) 6	(6–) 12	3
Deep tendon reflexes	(6–) 12	6 (–12)	(3–) 6	(6–) 12	3
Paraclinical examinations (recommended intervals in months)
Nerve conduction studies	12 (–24)	6 (–12)	6	–	6
Quantitative sensory testing	(6–) 12	6 (–12)	(6)	–	6
Skin conductance tests	(6–) 12	6 (–12)	(6)	–	6
Schellong’s test	(6–) 12	6 (–12)	6	–	3
Clinical scores and questionnaires (recommended intervals in months)
NIS	12	6 (–12)	(3–) 6	(6–) 12	3
PND/Coutinho stages	12	6 (–12)	(3–) 6	(6–) 12	3
COMPASS-31	12	6 (–12)	(3–) 6	(6–) 12	3
R-ODS	–	6 (–12)	6	(6–) 12	6
Norfolk QoL	–	6 (–12)	6	(6–) 12	6

Summarized recommendations for examination intervals in the different disease stages. Pre-symptomatic carriers should at least be monitored once per year depending on the physician’s discretion and their own preference. Symptomatic patients are recommended to be seen every 6 months. With a growing disease burden, patients in stage 2 might need a tighter follow-up even, whereas with the loss of mobility, stage 3 patients might prefer not to come to the center more often than once per year. Independent from the disease stage, any shift in treatment modalities always requires a tight follow-up at least every 3 months until the disease progression is halted. Representing the first symptoms in an early stage 1, sensory and autonomic tests are of special interest, while nerve conduction studies can still be normal. With disease progression, however, quantitative sensory testing loses its specificity