Neuropsychiatric syndrome with myoclonus after SARS-CoV-2 infection in a paediatric patient.

We report the case of a 12-years-old patient who subacutely developed a positive and negative myoclonus of limbs and face, drowsiness and memory deficits after getting infected by SARS-CoV-2. On admission, nasopharyngeal swab for SARS-CoV-2, brain and spinal MRI with and without contrast, EEG, chest X-ray and abdominal ultrasound were negative. CSF physical-chemical examination, culture, PCR testing for SARS-CoV-2 and other pathogens, and oligoclonal IgG bands were negative as well. A full panel blood test, including clotting, autoimmunity and paraneoplastic blood studies, did not show any alteration. The neuropsychological examination showed an impairment in memory, visual-motor coordination, inductive reasoning skills, attention, and concentration. The patient was first treated with clonazepam and then with intravenous methylprednisolone for five days, with poor response. For this reason, he then received a cycle of IVIG, thus reaching a gradual and complete recovery. To date, this is the first case of a COVID-19 associated myoclonus affecting a paediatric patient.

Neurological Complications Of Hand, Foot And Mouth Disease In Children: A Review.

BACKGROUND: Hand-Foot-and-Mouth disease (HFMD) is a viral illness commonly seen in young children, characterized by fever, vomiting, ulcerative lesions in oral mucosa, and vesicles on hands and feet. The early symptoms resolve but sometimes, it leads to more harsh neurological complications and even death. Therefore, the objective of this review was set to provide an overview of the symptoms, pathogenic agents, and treatment of neurological complications associated with HFMD. METHODS: We reviewed literature from PubMed and Science Direct covering at least one of our objectives from inception to 4th March 2018. RESULTS: This review represents 6 countries including China, Vietnam, Cambodia, South Korea, Taiwan, and Australia. Fifteen studies with a total of 1043 patients were included. The majority of HFMD cases with neurological complications were reported in China, predominance in boys as compared to girls, with 97% cases under 15 years of age. Meningoencephalitis and brainstem encephalitis contributed 70% of all neurological complications related to HFMD. Human Enterovirus71 genotype C, especially C4a was a causative agent associated with severe complications. Among symptoms, fever, vomiting, myoclonic jerks or seizure, headache, convulsion, and rashes were reported in almost all neurological complications. The common and supportive treatments were the administration of intravenous immunoglobulin and glucocorticoid therapies. CONCLUSIONS: Early detection and appropriate treatment of severe neurological complications can minimize the risk of adverse health outcomes. Evidence based clinical practice guidelines for early detection and treatment would be significant in the management of these devastating neurological complications.

Diaphragmatic myoclonus due to SARS-CoV-2 infection.

A wide range of neurological signs and symptoms have been associated with SARS-CoV-2 infection. In the present report, we described two Italian patients diagnosed with diaphragmatic myoclonus after COVID-19. In both cases, mild lymphocytosis at cerebrospinal fluid analysis and no structural brain changes were reported. The pathophysiological origin of the myoclonus in the two cases was different. In case 1, electroencephalogram did not reveal any cortical correlates and brain imaging of the spine was unremarkable, while in case 2, cortical origin of myoclonus was demonstrated. With the present two cases, we confirm and extend the neurological manifestations of SARS-CoV-2 infection.

Molecular epidemiology of enteroviruses associated with severe hand, foot and mouth disease in Shenzhen, China, 2014-2018.

In this study, we investigated the epidemiology and molecular characteristics of enteroviruses associated with severe hand, foot and mouth disease (HFMD) in Shenzhen, China, during 2014-2018. A total of 137 fecal specimens from patients with severe HFMD were collected. Enterovirus (EV) types were determined using real-time reverse transcription polymerase chain reaction (RT-PCR), RT nested PCR, and sequencing. Sequences were analyzed using bioinformatics programs. Of 137 specimens tested, 97 (70.8%), 12 (8.8%), and 10 (7.3%) were positive for EV-A71, coxsackievirus A6 (CVA6), and CVA16, respectively. Other pathogens detected included CVA2 (2.9%, 4/137), CVA10 (2.9%, 4/137), CVA5 (0.7%, 1/137), echovirus 6 (E6) (0.7%, 1/137) and E18 (0.7%, 1/137). The most frequent complication in patients with proven EV infections was myoclonic jerk, followed by aseptic encephalitis, tachypnea, and vomiting. The frequencies of vomiting and abnormal eye movements were higher in EV-A71-infected patients than that in CVA6-infected or CVA16-infected patients. Molecular phylogeny based on the complete VP1 gene revealed no association between the subgenotype of the virus and disease severity. Nevertheless, 12 significant mutations that were likely to be associated with virulence or the clinical phenotype were observed in the 5'UTR, 2Apro, 2C, 3A, 3Dpol and 3'UTR of CVA6. Eight significant mutations were observed in the 5'UTR, 2B, 3A, 3Dpol and 3'UTR of CVA16, and 10 significant mutations were observed in the 5'UTR, VP1, 3A and 3Cpro of CVA10. In conclusion, EV-A71 is still the main pathogen causing severe HFMD, although other EV types can also cause severe complications. Potential virulence or phenotype-associated sites were identified in the genomes of CVA6, CVA16, and CVA10.

Periodic myoclonus due to cytomegalovirus encephalitis in a patient with good syndrome.

BACKGROUND: Good syndrome (GS) refers to a constellation of thymoma and adult-onset immunodeficiency characterized by low or absent B cells, hypogammaglobulinemia, and variable defects in cell-mediated immunity with an inverted CD4/CD8(+) T-cell ratio. Patients may develop severe or chronic infections as a result of this immunodeficiency. OBJECTIVE: To describe a patient with GS who developed cytomegalovirus (CMV) encephalitis and showed a periodic electroencephalographic pattern and myoclonus. DESIGN: Case report. SETTING: Outpatient neurology clinic at a university medical center. Patient A 64-year-old man who developed periodic myoclonus involving the right half of his body and the left arm. RESULTS: Five years previously, the patient had undergone resection of a pathologically confirmed epithelial thymoma. Quantitative CMV polymerase chain reaction of the serum and cerebrospinal fluid showed strongly positive results (1:10 000), allowing the diagnosis of CMV encephalitis. CONCLUSIONS: To the best of our knowledge, myoclonus and periodic electroencephalographic pattern have not previously been reported in CMV encephalitis. Opportunistic CMV infection should be considered early in the evaluation of patients with GS or a history of thymoma who develop unusual neurological symptoms.

Neurological manifestations of dengue virus infection.

AIM: Paucity of studies on neurological manifestations in dengue virus infection prompted this study. We aim to correlate clinical, radiological and neurophysiological changes in dengue patients with neurological manifestations. METHOD: Consecutive IgM seropositive dengue patients admitted in neurology ward during 2003-2005 have been prospectively evaluated. They were subjected to detailed clinical evaluation, blood counts, coagulation profile, serum chemistry including creatine kinase (CK), cerebrospinal fluid (CSF), cranial CT and/or MRI, electroencephalogram (EEG), nerve conduction and needle electromyography (EMG). RESULTS: There were 17 patients, aged 5 to 56 years; 11 presented with encephalopathy and 6 with acute motor weakness. In the patients with encephalopathy, seizures were present in 3, myoclonus in 1, CSF pleocytosis and EEG slowing in 8 each and globus pallidus and thoracic spinal cord involvement on MRI in 1 patient each. In the pure motor weakness group, CK was elevated in 5 and EMG and muscle biopsy were consistent with myositis in 1 patient each. The patients with pure motor weakness improved completely but in the encephalopathy group 3 died, 2 had partial, 1 poor and 5 complete recovery by 1 month. CONCLUSION: Dengue patients presenting with encephalopathy had more severe illness and worse outcome compared to acute pure motor weakness.

Detection of herpesvirus-6A in a case of subacute cerebellitis and myoclonic dystonia.

This is a case study of a child who developed roseola infantum first, then varicella, and was later affected by acute cerebellar syndrome, severe truncal ataxia, and myoclonic dystonia. Human herpesvirus 6 (HHV-6) A and B were detected in the cerebrospinal fluid (CSF) and peripheral blood, respectively, upon ataxia onset. The intricacy of this case suggests multifaceted conclusions ranging from the need for a multidirectional approach to neurological diseases, to confirmation of a more pronounced neurotropism of HHV-6A and a possible role of viruses in myoclonic dystonia syndrome, although this last hypothesis should be confirmed by larger studies.

Neurologic manifestations and outcome of West Nile virus infection.

CONTEXT: The neurologic manifestations, laboratory findings, and outcome of patients with West Nile virus (WNV) infection have not been prospectively characterized. OBJECTIVE: To describe prospectively the clinical and laboratory features and long-term outcome of patients with neurologic manifestations of WNV infection. DESIGN, SETTING, AND PARTICIPANTS: From August 1 to September 2, 2002, a community-based, prospective case series was conducted in St Tammany Parish, La. Standardized clinical data were collected on patients with suspected WNV infection. Confirmed WNV-seropositive patients were reassessed at 8 months. MAIN OUTCOME MEASURES: Clinical, neurologic, and laboratory features at initial presentation, and long-term neurologic outcome. RESULTS: Sixteen (37%) of 39 suspected cases had antibodies against WNV; 5 had meningitis, 8 had encephalitis, and 3 had poliomyelitis-like acute flaccid paralysis. Movement disorders, including tremor (15 [94%]), myoclonus (5 [31%]), and parkinsonism (11 [69%]), were common among WNV-seropositive patients. One patient died. At 8-month follow-up, fatigue, headache, and myalgias were persistent symptoms; gait and movement disorders persisted in 6 patients. Patients with WNV meningitis or encephalitis had favorable outcomes, although patients with acute flaccid paralysis did not recover limb strength. CONCLUSIONS: Movement disorders, including tremor, myoclonus, and parkinsonism, may be present during acute illness with WNV infection. Some patients with WNV infection and meningitis or encephalitis ultimately may have good long-term outcome, although an irreversible poliomyelitis-like syndrome may result.

The use of the ketogenic diet in a patient with subacute sclerosing panencephalitis.

OBJECTIVE: To report on the effects of the ketogenic diet on a 9-year-old boy with myoclonic jerks due to subacute sclerosing panencephalitis (SSPE). METHODS: A 9-year-old boy presented with progressively worsening myoclonus unresponsive to valproic acid and clonazepam. He was started on the ketogenic diet maintaining urine ketones at greater than 80 mg x dl(-1). RESULTS: Within 2 weeks of dietary initiation, myoclonic jerks stopped. Four weeks later he developed cognitive slowing. Results of electroencephalogram and cerebrospinal fluid analysis were consistent with SSPE. Three months after ketogenic diet initiation, myoclonic jerks reappeared and were refractory to treatment. CONCLUSION: The ketogenic diet may be useful in controlling, even temporarily, the myoclonic jerks of SSPE.

Early onset and rapidly progressive subacute sclerosing panencephalitis after congenital measles infection.

UNLABELLED: We report an 18-month-old girl with rapidly progressive subacute sclerosing panencephalitis, whose non immunised mother had measles at the time of delivery. The patient presented with repetitive episodes of myoclonic jerks of the head and arms, followed by a drop of head and trunk with frequent falls. EEG, CSF studies and MRI confirmed the diagnosis. Despite therapy with isoprinosine and valproate, seizure activity continued and she became vegetative within 2 months, with severe spasticity and swallowing difficulties, and died at the age of 28 months. Early age of onset and rapid progression were most likely related to haematogenous in utero acquisition of the measles virus prior to delivery, as well as immaturity of neuronal and immune systems. CONCLUSION: this case emphasises the importance of a high measles vaccine coverage in the population in order to prevent the risk of disease in general and, in particular, gestational measles.

Progressive myoclonic ataxia and JC virus encephalitis in an AIDS patient.

A case of progressive myoclonic ataxia in an AIDS patient is described, which evolved over a 13 month period. The ataxia persisted as the only clinical finding for several months before the appearance of a severe tetraparesis and cachexia. Throughout the clinical progression, magnetic resonance imaging (MRI) revealed the presence of bilateral, progressive, isolated, and symmetrical lesions involving the red nuclei, subthalami, thalami, lenticular nuclei, and primary motor cortices. Neuropathological examination, supplemented by in situ hybridisation for JC virus DNA, confirmed that the lesions were those of progressive multifocal leucoencephalopathy (PML). The exceptional clinical presentation of PML in this case is the first report of progressive myoclonic ataxia caused by PML. The selective nature of the lesions confirms the role of the dentato-rubral-thalamo-cortical tract in the pathogenesis of progressive myoclonic ataxia. The atypical MRI findings further emphasise the need for expanded diagnostic criteria for PML in AIDS patients and support the use of more aggressive diagnostic methods as new treatments become available.

Palatal myoclonus in postinfectious opsoclonus myoclonus syndrome : a case report.

An adult male presenting with acute onset opsoclonus, myoclonus and cerebellar ataxia is being reported. Patient had myoclonus involving limbs and palate. There are only a few reported cases associated with palatal myoclonus. Patient showed gradual spontaneous recovery. Possibility of underlying malignancy was excluded by detailed investigations.

Multiple oral procedures performed on a dog with distemper myoclonus.

A 3-year-old male mixed-breed dog was treated for an oral defect along the rostral portion of the hard palate and an oronasal fistula secondary to a severe mandibular brachygnathism and persistent canine distemper virus associated myoclonus. This report describes the treatment using a bilateral radical premaxillectomy, oronasal fistula repair, and pulpotomy.