A Descending Cranial Nerve Palsy During the Christmas Holidays

Abstract

A previous healthy 11-year-old child with a 24-hour history of asthenia, diplopia, dysarthria, dysphonia, swallowing difficulty, and vomiting was admitted to our institution on Christmas eve. The child complained of perioral paresthesias and blurry vision 2 days prior to admission and an unusual tiredness after skiing on the day of admission. He presented with a temperature of 36.8°C, blood pressure 100/70 mm Hg, pulse 80 beats/min, respiratory rate 20/min, and oxygen saturation 99% on room air. He was awake, well oriented and his physical examination was within normal limits. Neurological examination showed bilateral ophthalmoplegia with unreactive pupils, dysphonia, dysphagia with minimal limb weakness with associated hyporeflexia. Sensation was maintained, but he presented with urinary retention described as a poor urinary stream with intermittent flow, straining, and a sense of incomplete voiding and hesitancy. A complete blood count, electrolytes, arterial blood gas, C-reactive protein and herpes virus 1 and 2 (HSV-1 and HSV-2), Epstein-Barr virus (EBV) antibodies were within the normal range. Cerebrospinal fluid (CSF) examination was unremarkable (glucose 81 mg/dL, normal values 40-70), protein 20 mg/dL (normal values 15-45), lactate 11.7 mg/dL (normal values 10.8-19.8), and no erythrocytes or leucocytes. Polymerase chain reaction (PCR) for neurotropic viruses (human herpes virus 6, human herpes virus 7, human herpes virus 8, adenovirus, Parvovirus, EBarrV, herpes simplex virus 1 and 2, and varicella-zoster virus), cultures for meningococcus and fungi, and serology for Borrelia, Chlamydia Pneumoniae, enterovirus, influenza A and B, metapneumovirus, morbillivirus, and Mycoplasma Pneumoniae were all negative. The electroencephalography (EEG) was also normal. Intoxication or snakebite were excluded by medical history, a detailed neurological examination, and laboratory tests (urine and plasma toxicology screen for amphetamines, cannabinoids, cocaine, opiates, phencyclidine,alcohol, benzodiazepines and tricyclic antidepressants). There was no history of ingestion of homemade or rotten food that could contain botulinum spore or toxins. Twenty-four hours after admission, the patient developed respiratory failure and areflexia of the upper limbs requiring mechanical ventilation and admission to the pediatric intensive care unit (PICU). Repeat CSF examination showed normal glucose, protein and lactic acid, negative bacterial cultures, and PCR for viruses. No albuminocytologic dissociation was observed. Under the working diagnosis of a Miller-Fisher syndrome (MFS), the child was treated with intravenous immunoglobulin G (IgG) infusion (400 mg/kg) for 5 days with no clinical response, followed by 5 cycles of plasmapheresis. The child gradually recovered and it was possible to perform electromyography and nerve conduction studies (EMG/NCSs) which showed a normal conduction pattern. The edrophonium test, antibodies to voltage-gated calcium channels, to acetylcholine receptors (AChRs), and anti-ganglioside autoantibodies (IgG and IgM for GM1, GM2, GD1a, GD1b, and GQ1B) were also negative. A diagnostic result was received.