Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Acute oropharyngeal palsy with localized sensory impairment resembling symptom distribution of acute pharyngeal-cervical-brachial variant in a patient with Guillain-Barré syndrome

Nobuhito Nakajima, M.D.1), Masayuki Ueda, M.D., Ph.D.2), Koichi Nomura, M.D.1), Susumu Kusunoki, M.D., Ph.D.3) and Yasuo Katayama, M.D., Ph.D.2)

1)Department of Neurology, Kitamurayama Public Hospital
2)Department of Neurology, Nippon Medical School
3)Department of Neurology, Kinki University School of Medicine

We present the case of a 40-year-old woman who experienced dysarthria and, numbness in her upper extremities and posterior region of her neck. Upon admission to our hospital, neurological examination revealed rhinolalia aperta and an incomplete palatoplegia; however, muscle strength in the neck and limbs was satisfactorily preserved, tendon reflexes were normal, and pathological reflexes were not observed. Cerebrospinal fluid and electrophysiological test results were also normal. On day 3 of hospitalization, a slight backflow of fluid into the nasal cavity was observed upon deglutition, and vibration perception was also impaired in the bilateral arms. Her serum tested positive for immunoglobulin G antibodies against such gangliosides as GT1a, GQ1b, GT1b, and GD1a. Despite normal tendon reflexes, she was diagnosed with a subtype of Guillain-Barré syndrome (GBS), and was treated with intravenous immunoglobulin therapy. Subsequently, her symptoms improved. Due to the combination of oropharyngeal palsy and sensory impairment, it was more likely the GBS subtype in this patient was acute oropharyngeal palsy (AOP) rather than pharyngeal-cervical-brachial (PCB) variant; though interestingly, the patient's sensory disturbance was limited to the posterior neck and upper extremities, which resembles the distribution of motor symptoms in PCB variant. The present case was a rare and important phenotype, demonstrating diversities of GBS variants. We also believe that GBS subtypes may represent a continuum of pathological conditions and not just one static condition. However, further studies involving serological characteristics of anti-ganglioside antibodies and clinical features for GBS are needed to clarify this possibility.
Full Text of this Article in Japanese PDF (944K)

(CLINICA NEUROL, 53: 630|633, 2013)
key words: Guillain-Barré syndrome, acute pharyngeal-cervical-brachial variant of GBS, acute oropharyngeal palsy

(Received: 28-Dec-12)