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Myoclonus in the context of COVID-19 is an increasingly recognized condition. The occurrence in an ICU context in hypoxic patients, with metabolic disorders, taking several types of medication, makes difficult to establish a precise cause. Also, the implication of SARS-CoV-2 by direct invasion of the CNS or by immune-mediated phenomena is not yet clear. Currently, a dozen of cases of myoclonus as a predominant clinical manifestation, immune-mediated by SARS-Cov-2 are published. In all these cases, myoclonus was preceded by respiratory or other suggestive symptoms (e.g., anosmia) for this infection making straightforward the causal link. We describe a case of an isolated generalized myoclonus without other clinical complaints nor chest CT scan abnormalities nor SARS-CoV-2 RNA detection on nasopharyngeal swabs and on the CSF, as a para-infectious phenomenon of COVID-19 infection with excellent response to steroids perfusion. This challenging diagnosis was made upon confirmation of seroconversion (serology was negative at admission, then positive for IgM at day 6, then for both IgM and IgG at day 10) underlying that repeating serology is a diagnostic key to capture a similar findings., (© 2021. Fondazione Società Italiana di Neurologia.)

Chorea paralytica (or chorea mollis) is a very rare variant of Sydenham's chorea, characterized by a profound hypotonia, resulting in severe disability. Given the rarity of this condition, data on its prognosis are lacking. Most reports suggest that the delay from onset to recover total autonomy is long, usually several weeks to months which strongly affects the quality of life of these children. We report a videotape case of a 14-year-old girl, who became rapidly bedridden because of severe generalized chorea paralytica. Her clinical picture was totally improved 7 days only after initiation of an "aggressive" treatment, combining steroid pulse, haloperidol and long-term penicillin G, with no relapse after 4-year follow-up. We believe that the best care of this rare and severe form of Sydenham's chorea, should combine pathophysiological treatment with corticosteroids, preferably by pulse-therapy, symptomatic antichoreic treatment by neuroleptics, associated with a long-term antibiotic use to reduce recurrence risk.

Nontraumatic subarachnoid hemorrhage is a relatively rare disease, typically secondary to a ruptured aneurysm. We report the case of a 23-year-old patient who developed a subarachnoid hemorrhage caused by extensive cerebral venous thrombosis due to a factor V Leiden mutation. Cerebral venous thrombosis is an uncommon etiology of subarachnoid hemorrhage. This raises diagnostic difficulties and a therapeutic dilemma regarding the use of anticoagulants., (Copyright © 2012. Published by Elsevier Masson SAS.)

Benign myoclonic epilepsy of infancy is a rare idiopathic generalized epileptic syndrome occurring below the age of 3 years. Although benign outcome is presumed, some recent studies suggest less favorable outcome. A 14-year-old boy had a history of repeated episodes of myoclonic jerks of the shoulders and upper limbs in infancy (age 5 months). An ictal electroencephalogram indicated generalized spike-wave discharges associated with the myoclonic seizures, and the diagnosis of benign myoclonic epilepsy of infancy was made. Valproate treatment resulted in control of the myoclonic seizures, and the drug was withdrawn when the patient was 5 years of age. At the age of 10, he presented with episodes of eyelid jerks associated with brief lapses in concentration triggered by sunlight. Electroencephalography revealed photosensitivity and a pattern of eye-closure sensitivity. These features were compatible with the diagnosis of eyelid myoclonia with absences, or Jeavons syndrome. Lamotrigine eliminated the seizures. The evolution of benign myoclonic epilepsy of infancy to eyelid myoclonia with absences has been reported in one other case. A possible continuum of myoclonic epileptic syndromes, mediated by a common genetic abnormality, suggests the need for longer monitoring of patients with benign myoclonic epilepsy of infancy., (Copyright 2010 Elsevier Inc. All rights reserved.)

Diabetic patients during hyperglycaemic crises may present a rare syndrome characterised by a typical triad: unilateral involuntary movements (hemichoreahemiballism), radiological contralateral striatal abnormality, and rapid resolution of symptoms after glycae - mic correction. This study reports a series of patients showing less usual aspects and also discusses the pathophysiology of this clinical-radiological syndrome. We included in this study four patients presenting choreic or ballic involuntary movements and in whom aetiological assessment revealed frank non-ketotic hyperglycaemia, without other abnormalities that could explain the movement disorder. All the patients underwent CT or MR brain imaging. The typical triad was present in only one case. Less classical aspects were more frequently found: movement disorders revealed diabetes in two patients and one patient had generalised chorea and strictly normal neuroimaging. Correction of blood glucose was not sufficient to improve symptoms in two cases. In one, abnormal movements persisted despite treatment with tetrabenazine. The clinical, radiological and outcome spectrum of the syndrome of chorea-ballismus induced by non-ketotic hyperglycaemia is heterogeneous and not restricted to a typical triad.