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1.
J Pediatr Hematol Oncol ; 45(3): 152-154, 2023 04 01.
Article in English | MEDLINE | ID: covidwho-2291742

ABSTRACT

Opsoclonus myoclonus ataxia syndrome (OMAS) is a rare disorder that causes significant neurodevelopmental sequelae in children. Approximately half of pediatric OMAS cases are paraneoplastic, typically associated with localized neuroblastic tumors. Since early persistence or relapse of OMAS symptoms is common even after tumor resection, OMAS relapses may not routinely prompt reevaluation for recurrent tumors. We report a 12-year-old girl with neuroblastic tumor recurrence associated with OMAS relapse a decade after initial treatment. Providers should be aware of tumor recurrence as a trigger for distant OMAS relapse, raising intriguing questions about the role of immune surveillance and control of neuroblastic tumors.


Subject(s)
Opsoclonus-Myoclonus Syndrome , Female , Humans , Child , Opsoclonus-Myoclonus Syndrome/etiology , Opsoclonus-Myoclonus Syndrome/therapy , Neoplasm Recurrence, Local , Ataxia/therapy , Ataxia/complications
2.
J AAPOS ; 27(2): 110-112, 2023 04.
Article in English | MEDLINE | ID: covidwho-2220850

ABSTRACT

Opsoclonus-myoclonus-ataxia syndrome (OMAS) in children is most often of paraneoplastic origin, but it can also result from infectious processes, toxic and metabolic disorders, and organic events that cause damage to the brainstem or cerebellum. Post-vaccination OMAS has also been reported. We report the case of a 15-year-old girl who developed OMAS 24 hours after her first dose of mRNA COVID-19 (BioNTech) vaccine.


Subject(s)
COVID-19 Vaccines , COVID-19 , Opsoclonus-Myoclonus Syndrome , Adolescent , Female , Humans , Ataxia , Cerebellum , COVID-19/complications , COVID-19 Vaccines/adverse effects , Opsoclonus-Myoclonus Syndrome/etiology
3.
J Med Case Rep ; 16(1): 437, 2022 Nov 16.
Article in English | MEDLINE | ID: covidwho-2113828

ABSTRACT

INTRODUCTION: Miller-Fisher Syndrome (MFS) is a variant of Guillain-Barré syndrome (GBS), an acute immune-mediated neuropathy, which manifests as a rapidly evolving areflex motor paralysis. This syndrome presents as a classic triad: ophthalmoplegia, areflexia, and ataxia. MFS is usually benign and self-limited. CASE REPORT: A Caucasian patient was admitted to our hospital with the flu, loss of bilateral strength in the lower limbs and upper limbs and sudden-onset ataxia 7 days after receiving a first dose of the Oxford/AstraZeneca COVID-19 vaccine. On neurological examination, the patient had Glasgow Coma Scale score of 15, with absence of meningeal signs; negative Babinski sign; grade 2 strength in the lower limbs and grade 4 strength in the upper limbs; axial and appendicular cerebellar ataxia; and peripheral facial diparesis predominantly on the right, without conjugate gaze deviation. Cerebrospinal fluid (CSF) was collected on admission, and analysis revealed albuminocytological dissociation with CSF protein of 148.9 mg/dL; leukocytes, 1; chlorine, 122; glucose, 65 mg/mL; red cells, 2; and non-reactive venereal disease research laboratory test result. The COVID-19 IgG/IgM rapid immunological test was negative. Electroneuromyography revealed a recent moderate-grade and primarily sensory and motor demyelinating polyneuropathy with associated proximal motor block. DISCUSSION AND CONCLUSION: Miller-Fisher Syndrome may be related to events other than infections prior to neuropathy, as in the case reported here. The patient presented strong correlations with findings for MFS reported in the literature, such as the clinical condition, the results of electroneuromyography, and results of the CSF analysis typical for MFS. When treatment was provided as proposed in the literature, the disease evolved with improvement. Ultimately, the diagnosis of incomplete MFS was made, including acute ataxic neuropathy (without ophthalmoplegia).


Subject(s)
COVID-19 , Miller Fisher Syndrome , Ophthalmoplegia , Humans , Miller Fisher Syndrome/diagnosis , COVID-19 Vaccines , Ophthalmoplegia/etiology , Ataxia/complications
4.
Curr Med Res Opin ; 38(12): 2109-2118, 2022 Dec.
Article in English | MEDLINE | ID: covidwho-2087497

ABSTRACT

The COVID-19 pandemic caused by SARS-CoV2 has raised several important health concerns, not least increased mortality and morbidity. SARS-CoV2 can infect the central nervous system via hematogenous or transneuronal routes, acting through different receptors including ACE2, DPP4, and neuropilin 1 and cause several issues, including the focus here, cerebellitis. The cerebellum is an essential part of the CNS located adjacent to the brainstem with a complex micro and macroscopic structure. The cerebellum plays several physiological roles, such as coordination, cognition, and executive functioning. Damage to the cerebellum can lead to incoordination and ataxia. In our narrative review, we searched different databases from 2021 to 2022 with the keywords cerebellum and COVID-19; 247 studies were identified and reviewed, focusing on clinical studies and excluding non-clinical studies; 56 studies were finally included for analysis. SARS-CoV2 infection of the cerebellum can be seen to be assessed through many methods such as MRI, PET, CT, postmortem studies, and histological findings. These methodological studies have demonstrated that cerebellar infection with COVID-19 can bring about several sequelae: thrombosis, microbleed, hemorrhage, stroke, autoantibody production, ataxia, and widespread inflammation in the cerebellum. Such central effects are likely to exacerbate the known multiorgan effects of SARS-CoV2 and should also be considered as part of disease prognosis.


Subject(s)
COVID-19 , Humans , COVID-19/complications , Pandemics , SARS-CoV-2 , RNA, Viral , Ataxia/etiology
5.
BMJ Case Rep ; 15(10)2022 Oct 03.
Article in English | MEDLINE | ID: covidwho-2064085

ABSTRACT

We present a woman in her 40s who arrived at the emergency room with hypertension and optic ataxia. Her medical history is only relevant for obesity. Her lumbar puncture revealed high intracranial pressure and lymphocytic pleocytosis, and her neuroimaging tests, including angiography and venography, were normal. The patient improved after a cerebrospinal fluid drainage with a lumbar puncture, and her clinical manifestations resolved in parallel to the lymphocytic pleocytosis.The patient was diagnosed with a syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis and fully recovered 21 days after her discharge.


Subject(s)
Lymphocytosis , Nervous System Diseases , Ataxia/etiology , Female , Humans , Leukocytosis , Lymphocytosis/diagnosis , Syndrome
6.
Ophthalmic Genet ; 43(4): 543-549, 2022 08.
Article in English | MEDLINE | ID: covidwho-2062673

ABSTRACT

BACKGROUND: Coats plus syndrome or cerebroretinal microangiopathy with calcifications and cysts (CMCC) is an exceedingly rare autosomal recessive disorder that predominantly affects the microvasculature in the retina, brain, bones, and gastrointestinal system. Unlike Coats disease, CMCC is bilateral and affects multiple organ systems. MATERIALS AND METHODS: Case report. RESULTS: We report the case of two brothers with Coats Plus syndrome who presented with variable phenotypic expression. One sibling (Patient 1) was thought to have atypical retinopathy of prematurity and was only diagnosed with Coats plus after his older brother (Patient 2) presented with a seizure and a left upper extremity tremor at 4 years of age. The CTC1 mutation was confirmed in both patients. Aggressive treatment with laser photocoagulation and intravitreal bevacizumab dramatically improved the retinal vascular and exudative changes. CONCLUSION: Coats Plus syndrome can have a variable phenotypic presentation, including retinal vascular findings. This rare genetic disease should be in the differential diagnosis in patients who present with atypical retinal pathology, including Retinopathy of Prematurity, Familial Exudative Vitreoretinopathy, or Coats disease associated with non-specific multiorgan abnormalities.


Subject(s)
Central Nervous System Cysts , Leukoencephalopathies , Retinal Telangiectasis , Retinopathy of Prematurity , Ataxia , Brain Neoplasms , Calcinosis , Central Nervous System Cysts/genetics , Humans , Infant, Newborn , Laser Coagulation , Leukoencephalopathies/genetics , Male , Muscle Spasticity , Retinal Diseases , Retinal Telangiectasis/diagnosis , Retinal Telangiectasis/genetics , Retinal Telangiectasis/therapy , Retinopathy of Prematurity/diagnosis , Retinopathy of Prematurity/genetics , Seizures
7.
Brain Nerve ; 74(7): 861-866, 2022 Jul.
Article in Japanese | MEDLINE | ID: covidwho-1954939

ABSTRACT

The new coronavirus SARS-CoV-2 infection (COVID-19) has caused many casualties, mainly respiratory infections. However, it has also caused several neurological disorders including encephalitis/encephalopathy, demyelinating disease, Gullain-Barré sydrome etc. In addition, it has been clarified that movement disorders develop within a few days to weeks after infection. Vaccination for COVID-19 has progressed, but autoimmune neurological complications have also been reported. Although a causal relationship is suspected over time, this paper describes the pathophysiology of movement disorders such as myoclonus, opsoclonus, parkinsonism, and cerebellar ataxia, which are relatively common in COVID-19 infections, and their relevance to the SARS-CoV-2 vaccine.


Subject(s)
COVID-19 , Cerebellar Ataxia , Movement Disorders , Nervous System Diseases , Ataxia , COVID-19/complications , COVID-19 Vaccines , Humans , Movement Disorders/complications , Nervous System Diseases/etiology , SARS-CoV-2
8.
Intern Med ; 61(11): 1757-1760, 2022 Jun 01.
Article in English | MEDLINE | ID: covidwho-1951856

ABSTRACT

Guillain-Barré syndrome (GBS) has occasionally occurred in people who have received coronavirus disease 2019 (COVID-19) vaccines. Dysgeusia is rare symptom of GBS. We herein report a rare case of sensory ataxic GBS with dysgeusia just after the second dose of the Pfizer-BioNTech COVID-19 vaccine. Although autoantibodies against glycolipids were not detected, immunotherapy with intravenous immunoglobulin and methylprednisolone pulse therapy effectively ameliorated the symptoms. Our report suggests that the COVID-19 vaccine may induce various clinical subtypes of GBS, including a rare variant with sensory ataxia and dysgeusia.


Subject(s)
COVID-19 , Guillain-Barre Syndrome , Ataxia/etiology , BNT162 Vaccine , COVID-19/complications , COVID-19 Vaccines/adverse effects , Dysgeusia/etiology , Guillain-Barre Syndrome/diagnosis , Guillain-Barre Syndrome/drug therapy , Guillain-Barre Syndrome/etiology , Humans , RNA, Messenger , SARS-CoV-2 , Vaccination
9.
Medicine (Baltimore) ; 101(20): e29333, 2022 May 20.
Article in English | MEDLINE | ID: covidwho-1860982

ABSTRACT

RATIONALE: Miller Fisher syndrome (MFS) is a rare variant of Guillain-Barre syndrome, classically diagnosed based on the clinical triad of ataxia, areflexia, and ophthalmoplegia. MFS is usually preceded by viral infections and febrile illness; however, only a few cases have been reported after vaccinations. PATIENT CONCERNS: A 53-year-old hypertensive male presented with a 2-day history of progressive ascending paralysis of the lower limbs along with diplopia and ataxia, 8 days after the first dose of the Sinovac-Coronavac coronavirus disease 2019 (COVID-19) vaccination, with no prior history of any predisposing infections or triggers. DIAGNOSES: Physical examination showed moderate motor and sensory loss with areflexia in the lower limbs bilaterally. Routine blood investigations and radiological investigations were unremarkable. Cerebrospinal fluid analysis showed albuminocytologic dissociation and nerve conduction studies revealed prolonged latencies with reduced conduction velocities. The diagnosis of MFS was established based on the findings of physical examination, cerebrospinal fluid analysis, and nerve conduction studies. INTERVENTIONS: A management plan was devised based on intravenous immunoglobulins, pregabalin, and physiotherapy. However, due to certain socioeconomic factors, the patient was managed conservatively with regular physiotherapy sessions. OUTCOMES: Follow-up after 6 weeks showed remarkable improvement, with complete resolution of symptoms 10 weeks after the discharge. LESSONS: This case suggests that MFS is a rare adverse effect after COVID-19 vaccination and additional research is required to substantiate a temporal association. Further studies are needed to understand the pathophysiology behind such complications to enhance the safety of COVID-19 vaccinations in the future.


Subject(s)
COVID-19 Vaccines , COVID-19 , Miller Fisher Syndrome , Ataxia/chemically induced , COVID-19/diagnosis , COVID-19/prevention & control , COVID-19 Vaccines/adverse effects , Diplopia/chemically induced , Humans , Male , Middle Aged , Miller Fisher Syndrome/chemically induced , Miller Fisher Syndrome/diagnosis , Vaccination/adverse effects
10.
Neurol Sci ; 43(3): 1587-1592, 2022 Mar.
Article in English | MEDLINE | ID: covidwho-1603464

ABSTRACT

OBJECTIVE: This study aims to report the clinical heterogeneity of myoclonus in 6 patients infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). METHODS: Patient data were obtained from medical records from the University Hospital Dr. Josep Trueta, Girona, Spain. RESULTS: Six patients (5 men and 1 woman, aged 60-76 years) presented with different myoclonus phenotypes. All of them had a medical history of hypertension and overweight. The latency of myoclonus appearance ranged from 1 to 129 days. The phenotype most observed was generalized myoclonus. Special phenotypes such as painful legs and moving toes syndrome with jerking feet, Lazarus sign-like, action myoclonus/ataxia syndrome, and segmental myoclonus secondary to myelitis have been described too. Levetiracetam and clonazepam were medications most used successfully. Two patients died for complications not related to myoclonus. CONCLUSIONS: Our 6 cases highlight the heterogeneity of the clinical spectrum of myoclonus associated to COVID-19 (MYaCO). MYaCO pathogenesis is suspected to be due to an immune-mediated para- or post-infectious phenomenon; nevertheless, further research is needed to elucidate this hypothesis.


Subject(s)
COVID-19 , Cerebellar Ataxia , Myoclonus , Aged , Ataxia/complications , Cerebellar Ataxia/complications , Female , Humans , Male , Middle Aged , Myoclonus/complications , Myoclonus/etiology , SARS-CoV-2
12.
Pract Neurol ; 21(6): 466-467, 2021 Dec.
Article in English | MEDLINE | ID: covidwho-1526526
13.
Neurol Neurochir Pol ; 55(6): 549-558, 2021.
Article in English | MEDLINE | ID: covidwho-1463973

ABSTRACT

INTRODUCTION: Various neurological symptoms have been confirmed in the course of SARS-CoV-2 infection. Some of these are undoubtedly the aftermath of the developing inflammation and increased coagulation processes. However, there is also a group of symptoms that derive from possible autoimmune processes. These include primary hyperkinetic movement disorders such as myoclonus, ataxia, opsoclonus, and tremors. This study systematically reviews scientific reports presenting patients with hyperkinetic movement disorders as one of the neurological symptoms. MATERIAL AND METHODS: The available literature was systematically reviewed as per the recommendations of Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). The PubMed database was used in the range from 1 April, 2020, to 31 July, 2021. RESULTS: The PubMed database search identified 102 cases of patients with SARS-CoV-2 infection who developed hyperkinetic movement disorders. After excluding patients undergoing mechanical ventilation (n = 46) and a few other cases (n = 7), a group of 49 non-intubated patients was obtained. The mean age of the patients was 57.92 years, and 75.51% of the patients were male. The most common hyperkinetic movement disorders were ataxia (83.67%), myoclonus (67.35%), and tremor (30.61%). Symptoms appeared on average within two weeks of the first symptoms of infection. Most patients had symptoms significantly reduced or withdrawn (67.44%) or early partial improvement (30.23%). CONCLUSIONS: Based on the meta-analysis, it can be concluded that hyperkinetic movement disorders in the course of SARS-CoV-2 infection are an early symptom with a potential autoimmune background. They have a good prognosis with the applied treatment. Further observations are needed to determine their frequency and the most effective methods of treatment.


Subject(s)
COVID-19 , Cerebellar Ataxia , Ataxia , Humans , Hyperkinesis/etiology , Male , Middle Aged , SARS-CoV-2
15.
Neurol Neurochir Pol ; 55(3): 310-313, 2021.
Article in English | MEDLINE | ID: covidwho-1259716

ABSTRACT

AIM OF THE STUDY: The pandemic state of COVID-19 has resulted in new neurological post-infection syndromes. Recently, several papers have reported ataxia-myoclonus syndrome following SARS-CoV-2 infection. The aim of this study was to present our two cases and compare them to previously reported cases. MATERIALS AND METHODS: We present two video-accompanied new cases with ataxia-myoclonus syndrome following SARS-CoV-2 infection and discuss the studies published so far. RESULTS: Ataxia-myoclonus syndrome, isolated myoclonus, opsoclonus-myoclonus syndrome as post-COVID-19 syndrome following infection have been described in 16 patients (including our two cases). Patients have been treated with intravenous immunoglobulins and/or steroids except for 4 patients, which resulted in a significant improvement within 1-8 weeks. CONCLUSIONS AND CLINICAL IMPLICATIONS: The increasing number of patients with a similar symptomatology shows a significant relationship between COVID-19 infection and ataxia-myoclonus syndrome. The subacute onset of neurological symptoms after a resolved COVID-19 infection and prominent response to immunotherapy may suggest that the neurological manifestations are immune-mediated. Although recovery is highly possible, it may take several weeks/months, and clinicians should be aware of this diagnosis and the beneficial effects of immunological treatment administered as soon as possible.


Subject(s)
COVID-19 , Opsoclonus-Myoclonus Syndrome , Ataxia , COVID-19/complications , Humans , SARS-CoV-2 , Post-Acute COVID-19 Syndrome
19.
J Neurol ; 268(10): 3517-3548, 2021 Oct.
Article in English | MEDLINE | ID: covidwho-1092678

ABSTRACT

BACKGROUND: Since the beginning of the coronavirus disease 2019 (COVID-19) pandemic in December 2019, neurological manifestations have been recognized as potential complications. Relatively rare movement disorders associated with COVID-19 are increasingly reported in case reports or case series. Here, we present a case and systematic review of myoclonus and cerebellar ataxia associated with COVID-19. METHODS: A systematic review was performed according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guideline using the PubMed and Ovid MEDLINE databases, from November 1, 2019 to December 6, 2020. RESULTS: 51 cases of myoclonus or ataxia associated with COVID-19, including our case, were identified from 32 publications. The mean age was 59.6 years, ranging from 26 to 88 years, and 21.6% were female. Myoclonus was multifocal or generalized and had an acute onset, usually within 1 month of COVID-19 symptoms. Myoclonus occurred in isolation (46.7%), or with ataxia (40.0%) or cognitive changes (30.0%). Most cases improved within 2 months, and treatment included anti-epileptic medications or immunotherapy. Ataxia had an acute onset, usually within 1 month of COVID-19 symptoms, but could be an initial symptom. Concurrent neurological symptoms included cognitive changes (45.5%), myoclonus (36.4%), or a Miller Fisher syndrome variant (21.2%). Most cases improved within 2 months, either spontaneously or with immunotherapy. CONCLUSIONS: This systematic review highlights myoclonus and ataxia as rare and treatable post-infectious or para-infectious, immune-mediated phenomena associated with COVID-19. The natural history is unknown and future investigation is needed to further characterize these movement disorders and COVID-19.


Subject(s)
COVID-19 , Cerebellar Ataxia , Myoclonus , Ataxia/complications , Cerebellar Ataxia/complications , Female , Humans , Middle Aged , Myoclonus/etiology , SARS-CoV-2
20.
J Neurovirol ; 27(1): 26-34, 2021 02.
Article in English | MEDLINE | ID: covidwho-1046668

ABSTRACT

Opsoclonus-myoclonus-ataxia syndrome is a heterogeneous constellation of symptoms ranging from full combination of these three neurological findings to varying degrees of isolated individual sign. Since the emergence of coronavirus disease 2019 (COVID-19), neurological symptoms, syndromes, and complications associated with this multi-organ viral infection have been reported and the various aspects of neurological involvement are increasingly uncovered. As a neuro-inflammatory disorder, one would expect to observe opsoclonus-myoclonus syndrome after a prevalent viral infection in a pandemic scale, as it has been the case for many other neuro-inflammatory syndromes. We report seven cases of opsoclonus-myoclonus syndrome presumably parainfectious in nature and discuss their phenomenology, their possible pathophysiological relationship to COVID-19, and diagnostic and treatment strategy in each case. Finally, we review the relevant data in the literature regarding the opsoclonus-myoclonus syndrome and possible similar cases associated with COVID-19 and its diagnostic importance for clinicians in various fields of medicine encountering COVID-19 patients and its complications.


Subject(s)
Ataxia/physiopathology , COVID-19/physiopathology , Cough/physiopathology , Fever/physiopathology , Myalgia/physiopathology , Opsoclonus-Myoclonus Syndrome/physiopathology , SARS-CoV-2/pathogenicity , Adult , Anticonvulsants/therapeutic use , Ataxia/diagnostic imaging , Ataxia/drug therapy , Ataxia/etiology , Azithromycin/therapeutic use , COVID-19/complications , COVID-19/diagnostic imaging , Clonazepam/therapeutic use , Cough/diagnostic imaging , Cough/drug therapy , Cough/etiology , Dyspnea/diagnostic imaging , Dyspnea/drug therapy , Dyspnea/etiology , Dyspnea/physiopathology , Female , Fever/diagnostic imaging , Fever/drug therapy , Fever/etiology , Humans , Hydroxychloroquine/therapeutic use , Levetiracetam/therapeutic use , Male , Middle Aged , Myalgia/diagnostic imaging , Myalgia/drug therapy , Myalgia/etiology , Opsoclonus-Myoclonus Syndrome/diagnostic imaging , Opsoclonus-Myoclonus Syndrome/drug therapy , Opsoclonus-Myoclonus Syndrome/etiology , Oseltamivir/therapeutic use , SARS-CoV-2/drug effects , Valproic Acid/therapeutic use , COVID-19 Drug Treatment
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