On 17th of June, Liselotte Ruts obtained her doctor’s degree at the Erasmus Medical Center University Hospital, defending her thesis “Pain, autonomic dysfunction and course of disease in Guillain-Barré syndrome (GBS)". This thesis reflects the results of the GRAPH study, in which many neurologists in The Netherlands have participated. The main findings are summarized below.
In her study, she found that mildly affected GBS patients more often showed a preceding virological infection compared to severely affected GBS patients. This suggests that preceding infections may at least partially determine symptoms and severity of disease. Severely affected GBS patients more often showed autonomic dysfunction compared to mildly affected patients. Residual symptoms like functional disability, pain and fatigue appeared to be very common, not only in severely affected patients but also in Miller Fisher syndrome and in mildly affected GBS patients.
An important finding is the identification of factors that help to distinguish between GBS with treatment related fluctuations (GBS-TRF) and chronic inflammatory demyelinating polyneuropathy with acute onset (A-CIDP). The diagnosis of A-CIDP should be considered when “a patient with GBS” deteriorates again after 8 weeks from onset, or when deterioration occurs three times or more. Especially when the patients remains able to walk independently during the most severe phase of the disease, has no cranial nerve dysfunction and electrophysiological examination shows features of demyelinisation, it is likely that the patient has A-CIDP. In this case, maintenance treatment for CIDP should be considered.
It is likely that GBS, A_CIDP and CIDP are all within one spectrum ranging from very acute GBS on one side to a slowly progressive form of CIDP on the other side.
The GRAPH study also shows that pain is a frequent symptom in CIDP; in the acute phase 64% of the patients reported pain and in the majority of patients, the intensity of pain was moderate to severe. Pain was reported to occur already in the two weeks preceding weakness in 35% of the patients. Mainly radicular pain, painful par-/dysaesthesia and muscle pain were described in the acute phase. After 6 months, painful par-/dysaesthesia and muscle pain were predominantly present. It is likely that sensory nerve fiber involvement results in more pain.
Skin biopsy studies revealed that in GBS patients, distal and lumbar intraepidermal nerve fiber density (IENFD) of small diameter nerve fibers were lower in the acute phase as compared to controls. IENFD remained lower also at 6-month follow-up. Loss of small fibers was associated with the presence and intensity of neuropathic pain, autonomic dysfunction and –to some extent- with worse outcome. Research using skin biopsies may lead to ore insight into the pathophysiology of features leading to pain and autonomic dysfunction.
If you have a news item for the ISNO website, please mail us