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A 37 year-old female presented with 5-weeks of neck pain, proximal weakness and dysphonia. Exami- nation showed facial rash, proximal weakness, areflexia and distal sensory loss. She had a 6-year history of sensory neuropathy, treated functional B12 deficiency and fibromyalgia. Serum showed elevated CK (360U/L) and borderline positive Anti-Ro52. Electrophysiology showed worsening sensory neuropathy and new myopathic features. She deteriorated soon after receiving intravenous corticosteroids and immu- noglobulins. CK increased to 1796 U/L and she developed bulbar failure requiring respiratory support.Muscle biopsy electron microscopy showed lipid accumulation suggesting a metabolic disorder. Serum acylcarnitines and urinary organic acids were abnormal, consistent with Riboflavin Transporter Deficiency (RTD). Riboflavin 500mg three times daily was initiated, urinary organic acids normalised and the patient regained full-power with neurorehabilitation.RTD is a rare cause of peripheral and cranial neuropathy, with autosomal recessive inheritance due to variants in riboflavin transporter genes and onset up to young adulthood. Mutation carrier frequencies should generate 70 new cases per year worldwide, though only ~80 case-reports exist in the literature suggesting under-recognition. Riboflavin is a precursor for coenzymes important for carbohydrate, amino acid and lipid metabolism. Untreated, the disorder is fatal, though the majority of patients improve with Riboflavin supplementation.elena.purcaru@gmail.com

Guillain-Barre syndrome (GBS) is a heterogeneous disorder causing muscle weakness, sensory change, dysautonomia and often involving cranial neuropathies. An immune response to recent infection or to triggers such as vaccines, is thought to be responsible. Immunological cross-reaction with the peripheral nerve causes subsequent demyelinating or axonal damage. GBS is a continuous spectrum of disease rather than discrete phenotypes. Recognised GBS variants in order of frequency are: (1) classical; (2) Miller Fisher syndrome; (3) pharyngeal-cervical-brachial; (4) bifacial weakness with paraesthesia of limbs (BFP); and (5) paraparetic.1 At the time of publication, no reports of BFP variant GBS post COVID-19 vaccination have been described. We report five remarkably similar cases of BFP post-Vaxzevria. All of them presented to Wessex hospitals: Queen Alexandra Hospital, Portsmouth; Southampton General Hospital; Poole Hospital; and St Mary’s Hospital, Isle of Wight, within a 3-month period. A 66-year-old man developed interscapular back and leg pain, particularly severe at night, 1 week after his first dose of Vaxzevria. He had paraesthesia of both hands and feet and was unsteady. Ten days later, he developed bilateral facial weakness with numbness of the tongue and mouth. Examination revealed marked bilateral lower motor neuron (LMN) facial weakness. Tone, power and reflexes were normal in the limbs except absent right ankle jerk. Light touch and pinprick sensation was reduced symmetrically in both lower limbs to the knee and vibration to the ankles. His gait was ataxic. A 43-year-old man …