Background: The aim of this study was to further characterize the clinical phenotype of hepatitis E virus (HEV)-associated neuralgic amyotrophy (NA).Methods: Three patients with HEV-associated NA underwent clinical, electrodiagnostic, and ultrasound assessment.Results: In all patients, symptoms developed in several phases within a time span of 4-6 weeks, with three or more nerves involved. Symptoms were bilateral in two. In two patients, nerves of the trunk and the lower limb were affected as well. In one patient, three bouts occurred, each heralded by an increase in pain. In the other two, pain subsided quickly and nerve damage developed in two phases. Segmental enlargement with or without hourglass-like constrictions of the nerves was demonstrated by ultrasound in all. Conclusions:The multiphasic presentation, together with the extensive multi-nerve involvement, may reflect a severe and protracted inflammation of the nerves in HEVassociated NA. K E Y W O R D S clinical phenotype, hepatitis E virus infection, hourglass-like constriction, multiphasic presentation, neuralgic amyotrophy, Parsonage-Turner syndrome 1 | INTRODUCTION Neuralgic amyotrophy (NA) is an acute painful dysimmune neuropathy, typically affecting nerves originating from the brachial plexus. 1,2Lately, a specific association between hepatitis E virus (HEV) infection and NA has emerged. Since its first report in 2009, 3 an increasing number of cases have been published and a distinct clinical phenotype of HEV-associated NA has been described. [4][5][6][7] With the aim of further characterizing HEV-associated NA, we describe here 3 patients with an unusual multiphasic clinical presentation. | METHODSApproval was obtained from the Institutional Ethics Committee.The following criteria were used for the diagnosis of NA, (1) acute pain in the shoulder or arm, associated with weakness or sensory loss in the distribution of one or more peripheral nerves; (2) electrodiagnostic (EDX) proof of axon loss; (3) absence of other conditions explaining the symptoms. The diagnosis was further supported by imaging findings. Patients underwent clinical, EDX, and high-resolution ultrasound examination of affected nerves. The diagnosis of HEV infection associated with the acute stage of NA was based on elevated transaminase levels and HEV IgM antibody positivity.3 | RESULTS | Patient 1The 30-year-old man experienced an acute onset of intense pain in the left forearm, followed by loss of sensation on the lateral aspect of the left forearm (indicative of left lateral antebrachial cutaneous nerve lesion). Two weeks later, fever developed and the pain spread to the whole left arm and shoulder. The fever subsided within days, when he noticed the inability to flex the distal phalanx of his left thumb (presumed left partial anterior interosseous nerve lesion). At this point, he received a single dose of 2 mg of intramuscular betamethasone, leading to pain reduction. One month later (6 weeks after onset), fever lasting for 2-3 days developed again, the pain intensified and be...

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We report the association of ipsilateral trigemino-autonomic headache to a case of right-sided nuclear facial and abducens palsy (Gasperini syndrome), ipsilateral hypacusis and right hemiataxia, caused by the occlusion of the right anterior inferior cerebellar artery. Short-lasting attacks of mild to moderate ipsilateral fronto-periorbital head pain, accompanied by lacrimation and mild conjunctival injection during more severe attacks, were present from the onset of symptoms, with a gradual worsening over the next few months and remitting during naproxen therapy. Magnetic resonance imaging showed an infarct in the right cerebellar peduncle, extending toward the pontine tegmentum, also involving the ipsilateral spinal trigeminal nucleus and tract and the trigeminal entry zone. Gasperini syndrome may be accompanied by ipsilateral trigemino-autonomic head pain.

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