A report of a probable case of familial Guillain Barre syndrome
Two years later, her 3.5- year-old daughter was admitted to the Tabriz Children Hospital with lower limb weakness since the day before hospitalization while she had experienced an episode of gastroenteritis 10 days before. She was not able to stand or walk without aid and complained of lower limb and back pain with difficulty in swallowing. She was wheelchair bound in 2 days. Electrophysiological studies showed reduced motor nerve conduction velocities and F wave latencies were prolonged significantly in the lower limbs. Secondary axonal loss was also reported in the studies. These generalized motor dominant slowed conduction velocities with secondary axonal injury were compatible with demyelinating polyneuropathy. CSF analysis showed cytoprotein dissociation with 89 mg/dL protein concentration and no cells. Serological studies for C. jejuni infection were positive. Considering the patient's clinical course besides these paraclinical findings, the diagnosis was an acute inflammatory motor-dominant demyelinating polyneuropathy.\nShe was treated with a 5-day course of intravenous immunoglobulin (2 g/kg). No ventilation support was needed. On Day 14, she walked a 5-meter distance with aid. In 2 months, she could walk the same distance with no aid and she completely recovered in 5 months.\nHLA class II typing for DRB by PCR SSP method identified HLA- DRB1*11 (DRB3) and HLA- DRB1*03, which was identical in both cases. The HLA A2 and Cw2 antigens were also identical in the mother and daughter's HLA I typing.\nThe detailed nerve conduction velocity studies of the two cases are included in in next page.