Background. Amyotrophic lateral sclerosis (ALS) is a fatal disease whose diagnosis and early management can improve survival. The most used diagnostic criteria are the revised El Escorial criteria (rEEC) and Awaji criteria (AC). The comparison of their sensitivities showed contradictory results. Our study aimed to compare the sensitivities of these two criteria in the diagnosis of definite ALS, at first visit, in a Tunisian hospital cohort. Materials and Methods. This was a retrospective study including 173 patients diagnosed with ALS at the Department of Neurology of the Razi Hospital between January 2003 and April 2018.After studying the clinical features of the disease in our study population,each patient was categorized according to the rEEC and AC based on data collected in his medical record during his first visit to our department. Then, we compared the sensitivities of these two criteria in the diagnosis of definite ALS. Results. Our Tunisian cohort was characterized by a slower disease progression. The sensitivity of the AC (69.4%) was significantly higher than that of the rEEC (40.5%) ( p  < 0.001). When the clinical signs evolved for less than 6 months, the sensitivities were 61% for AC and 12% for rEEC ( p  < 0.001). After 24 months of disease progression, the sensitivities were 78.2% for AC and 69.1% for rEEC ( p  = 0.063). It was impossible to categorize seventeen patients by the two criteria. Conclusion. Our study demonstrated that patients in AC are more sensitive than rEEC in the early diagnosis of ALS in our Tunisian cohort. However, this superiority is gradually reduced during the evolution of the disease.

Background Polyradiculoneuropathy following infection with varicella zoster virus (VZV) is rare and most of the time, happens in the context of reactivation of latent VZV. We report a case of acute polyradiculoneuropathy following primary infection with VZV marked by atypical clinical features raising the hypothesis of a para-infectious disease. Case presentation We describe a 43-years-old male who developed ataxia, dysphagia, dysphonia, and oculomotor disorders (vertical binocular diplopia and bilateral ptosis) followed by quadriplegia with areflexia which occurred 4 days later. The patient had a history of varicella that occurred 10 days before the onset of these symptoms. Nerve conduction study revealed features consistent with an acute motor-sensory axonal neuropathy (AMSAN). Anti-ganglioside antibodies were negative. Based on clinical presentation and ancillary examination, we retain the Miller Fisher/Guillain-Barré overlap syndrome diagnosis. The patient was treated with high doses of methylprednisolone but the evolution of the disease was nevertheless marked by a complete recovery six weeks after onset of symptoms. Conclusion GBS following varicella is a rare but severe disease occurring most often in adults and marked by greater involvement of the cranial nerves. Its clinical features suggest that it is a para-infectious disease. Antiviral therapy has no effect on the course of the disease but its administration within the first 24 h after the onset of chickenpox in adults can prevent its occurrence.

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