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1.
Clinical Immunology ; Conference: 2023 Clinical Immunology Society Annual Meeting: Immune Deficiency and Dysregulation North American Conference. St. Louis United States. 250(Supplement) (no pagination), 2023.
Article in English | EMBASE | ID: covidwho-20241449

ABSTRACT

Introduction: COVID-19 related encephalitis has been reported in pediatric patients;however, there are no reports in patients with inborn errors of immunity (IEI). Activated PI3K Delta Syndrome (APDS) is a disease of immune dysregulation with immunodeficiency, autoimmunity, and abnormal lymphoproliferation resulting from autosomal dominant gain-offunction variants in PIK3CD or PIK3R1 genes. We investigate a family with APDS, one mother and three children, one of whom developed COVID-19 related encephalitis. Method(s): Patients were consented to an IRB-approved protocol at our institution. Medical records and detailed immunophenotyping were reviewed. Family members were sequenced for IEI with a targeted gene panel. Result(s): The index case is a 10-year-old female with a known pathogenic variant in PIK3CD (c.3061 G > A, p.Glu1021Lys), who contracted SARS-COV-2 despite one COVID-19 vaccination in the series. Her disease course included COVID-related encephalitis with cerebellitis and compression of the pons, resulting in lasting truncal ataxia and cerebellar mutism. At that time, the patient was not on immunoglobulin replacement therapy (IgRT), but was receiving Sirolimus. Besides the index case, 3 family members (2 brothers, 1 mother) also share the same PIK3CD variant with variable clinical and immunological phenotypes. All children exhibited high transitional B-cells, consistent with developmental block to follicular B cell stage. Increased non-class switched IgM+ memory B cells and skewing towards CD21lo B cell subset, which is considered autoreactive-like, was observed in all patients. Of note, the patient had low plasmablasts, but normal immunoglobulins. Of her family members, only one was receiving both sirolimus and IgRT. Conclusion(s): We describe a rare case of COVID-19-related encephalitis in a patient with inborn error of immunity while not on IgRT. This may indicate infection susceptibility because of a lack of sufficient immunity to SARS-CoV-2, unlike the rest of her family with the same PIK3CD variant.Copyright © 2023 Elsevier Inc.

2.
Neurology ; 93(23 Supplement 2):S29, 2022.
Article in English | EMBASE | ID: covidwho-2196702

ABSTRACT

Objective To describe clinical and paraclinical features of non-paraneoplastic NIFmediated disease associated with concurrent SARS-CoV-2 infection. Background Neurologic syndromes associated with neuronal intermediate filament (NIF) immunoglobulin G (IgG) most often are characterized by encephalopathy, cerebellar ataxia, or myelopathy. NIF-IgG has been strongly correlated with the presence of an underlying malignancy, with neuroendocrine tumors being most prevalent. Despite the intracellular target of this antibody, patients with NIF-IgG mediated disease tend to improve clinically with immunotherapy. While some cases have been described in a parainfectious context, this is the first such case in the context of a SARS-CoV-2 infection. Design/Methods NA. Results We reported a case of non-paraneoplastic NIF-mediated disease in the setting of SARS-CoV-2 infection. The patient presented with first time seizure. He was found to have frequent left temporal lobe spikes then two left temporal lobe seizures on neurotelemetry. Brain MRI displayed abnormal signal throughout the left hippocampus and mesial temporal lobe, without contrast enhancement. LP was subsequently performed. CSF showed elevated protein, 14-3-3, T-tau, interleukin 13, interleukin 2 receptor, and interleukin 6. The meningitis/encephalitis panel, and HSV-1/2 IgG were negative. Serum autoimmune encephalitis panel revealed a high-positive titer for anti-NIF 1:960, with concurrent NIF heavy chain cell-based assay positive. He improved with three days of IV steroids and treatment with levetiracetam and lacosamide. He has since been seizure free. Conclusions NIF-mediated diseases usually present with encephalopathy, cerebellar ataxia, or myelopathy and are generally seen in the setting of malignancy. Our case illustrated an example of NIF-mediated disease presenting as seizure in the setting of infection. This highlights the importance of consideration of parainfectious autoimmunity.

3.
Annals of Oncology ; 33(Supplement 9):S1607, 2022.
Article in English | EMBASE | ID: covidwho-2129914

ABSTRACT

Background: COVID-2019 had a dramatic impact on cancer care worldwide. There are numerous of vaccines developed or being developed in order to prevent the spread of the disease. A recombinant adenovirus-based vaccine, Gam-COVID-Vac (Sputnik V), has shown a favorable safety profile and efficacy in Phase 3 trial. Nowadays it is a main SARS-CoV-2 vaccine in Russia, but there is lack of information on its safety in cancer patients. We conducted a retrospective trial to assess safety of Sputnik V in adult patients with cancer. Method(s): we screened N.N. Blokhin NMRCO records for 01.2021-05.2022 timeframe and identified adult cancer patients vaccinated against SARS-CoV-2 with Sputnik V vaccine and contacted them to assess the tolerability and safety of the above mentioned vaccine. The patients were asked to report any new adverse events they experienced up to 28 days after the last dose of the vaccine. All the adverse events were recorded in the database and graded according to CTCAE criteria. Patients were specifically asked to report the following: pyrexia, asthenia, nausea, vomiting, local reactions, abdominal pain, muscle or joint pain and to report any other concerning symptoms. Symptoms were graded according to CTCAE4.03 criteria. Result(s): we identified 145 patients who received at least 1 dose of vaccine, safety data were available for 141 of them. Median age was 55 years (21-83), 70 (48.9%), 27 (19.2%), 21 (14.9%) and 19 (13.5%) patients had gynecologic, breast, genitourinary, gastrointestinal tumors, respectively;5 (3.5%) of patients had other types of tumors. Overall, 70 (49.6%) of patients experienced AE of any grade. Most common AEs were injection reactions (40.4%), pyrexia (24.1%), asthenia (22.0%) and arthralgia (13.5%), results are summarized in the table below. Few patients experienced grade 3-4 AEs, however 1 patient developed grade 4 cerebellar ataxia probably related to vaccination. Cancer type and active treatment were not predictors of AEs. [Formula presented]. Conclusion(s): Sputnik V vaccination appears to be safe and tolerable in patients with cancer, however additional studies should be conducted to assess efficacy and safety of the vaccine in cancer setting. Legal entity responsible for the study: The authors. Funding(s): Has not received any funding. Disclosure: A. Rumyantsev: Financial Interests, Personal, Invited Speaker: BIOCAD, AstraZeneca, Eisai, Pfizer, Merck, MSD, R-Pharm;Financial Interests, Personal, Stocks/Shares: AstraZeneca, Pfizer, Novartis, MSD. E. Glazkova: Financial Interests, Personal, Invited Speaker: Pfizer, AstraZeneca, MSD, Merck, Novartis, R-Pharm. A. Tryakin: Financial Interests, Personal, Invited Speaker: Bristol Myers Squibb, MSD, Eli Lilly, Merck, Amgen, Biocad;Financial Interests, Personal, Advisory Board: Bristol Myers Squibb, Astra Zeneca, Biocad;Financial Interests, Personal, Expert Testimony: R-pharm;Financial Interests, Institutional, Invited Speaker: MSD, BMS;Financial Interests, Personal and Institutional, Invited Speaker: Eli Lilly. A. Tyulyandina: Financial Interests, Personal, Funding: AstraZeneca, Roche, MSD, RUSSCO;Financial Interests, Personal, Speaker's Bureau: AstraZeneca, Eisai;Financial Interests, Personal, Invited Speaker: Roche, MSD, Pfizer, Tesaro, BIOCAD. All other authors have declared no conflicts of interest. Copyright © 2022

4.
Multiple Sclerosis Journal Conference: 38th Congress of the European Committee for Treatment and Research in Multiple Sclerosis, ECTRIMS ; 28(3 Supplement), 2022.
Article in English | EMBASE | ID: covidwho-2124558

ABSTRACT

The proceedings contain 1423 papers. The topics discussed include: diagnostic wandering in multiple sclerosis highlights the need for increased physicians alertness;limited diagnostic utility of serologic testing in the evaluation for multiple sclerosis and its mimics: a single-center observational study;potential use of biomarkers in the family members of new untreated relapsing-remitting multiple sclerosis for early diagnosis of multiple sclerosis;blood platelet RNA as biomarker for the detection of early stage multiple sclerosis;Leber hereditary optic neuropathy-plus syndrome mimicking a neuro-inflammatory disorder;anti-neurochondrin antibody as a potential biomarker in primary autoimmune cerebellar ataxia - a case report;validation of McDonald 2017 multiple sclerosis diagnostic criteria in patients with a first demyelinating event in Argentina;onset of various CNS inflammatory demyelination diseases following COVID-19 vaccinations;and motherhoodmotherhood choice in multiple sclerosis (MoMS) - feasibility and pilot study of two decision support programs choice in multiple sclerosis (MoMS) - feasibility and pilot study of two decision support programs.

5.
NeuroQuantology ; 20(6):9488-9497, 2022.
Article in English | EMBASE | ID: covidwho-2010508

ABSTRACT

Artificial intelligence (AI) is the emerging field to diagnose and analyze chronic illnesses like Cerebellar Ataxia (CA), Spinocerebellar Ataxia (SCA), and Parkinson's disease. AI technologies such as machine learning and deep learning assist many doctors, diagnosis departments, and medical personnel in identifying and analyzing neurological disorders. Nowadays, AI used in most of the health care applications. Our research paper proffers an innovative approach to classify neurological disorders with various Machine learning algorithms. Existing research works experimented with machine learning algorithms like Support Vector machine and KNN, the performance of these algorithm is good, when the data is less and binary classified. In the proposed work, we have applied SVM, KNN, Decision tree and AdaBoost algorithms on the CA Data set. The performance of proposed methods exhibit improved accuracy when compared with the existing works. The results of the proposed work are tabulated for comparative analysis. We found that the AdaBoost algorithm shows the better classification result for Cerebellar Ataxia disease severity.

6.
Journal of Clinical and Experimental Hepatology ; 12:S94-S95, 2022.
Article in English | EMBASE | ID: covidwho-1977442

ABSTRACT

Background and Aim: Metronidazole is commonly prescribed drug for amoebiasis and is usually well tolerated, and safe but can cause serious neurological adverse events including peripheral neuropathy which is relatively common but CNS toxicity is rare. We report a case of cerebellar ataxia who had taken metronidazole inadvertently for amoebic liver abscess. Case summary: Young male with history of toddy inking admitted for management of amoebic liver abscess. He was managed with percutaneous ain and intravenous metronidazole. He was discharged on oral metronidazole tablet for a total duration of 10 days. Due to COVID-19 pandemic, he did not turn up and continued taking metronidazole. Two months’ later patient presented with progressive slurring of speech and unsteady gait. On examination, cerebellar sign was present with normal motor and sensory system. Blood investigations including complete blood count, liver function test, kidney function test and thyroid profile were normal. Vitamin B12 and fasting blood sugar levels were normal. Non-contrast computed tomographic (NCCT) scan of brain was normal. Magnetic resonance imaging (MRI) scan of the brain showed areas of hyperintense signal change in dentate nucleus of cerebellum, two small foci in dorsal pons and splenium of corpus callosum with no restriction in T2 FLAIR, DWI and ADC sequences suggestive of interstitial edema. On stopping metronidazole, his sign and symptoms started waning and was symptom free after 10 days. Conclusions: Neurological toxicity may be related to prolonged administration, high doses, or high cumulative doses of metronidazole and prompt identification of neuropathy and cerebellar ataxia is essential to avoid permanent damage. Clinicians should avoid the use of metronidazole for more than 2 weeks in case of amoebic liver abscess.

7.
Neurology ; 98(18 SUPPL), 2022.
Article in English | EMBASE | ID: covidwho-1925572

ABSTRACT

Objective: We aim to report clinical characteristics of an extremely rare case of myelitis with Guillain-Barré syndrome (GBS) and cerebellar ataxia (CA) after COVID-19 infection. Background: There have been many reports about neurological complications following the world pandemic of COVID-19. We found about 100 GBS, 50 myelitis, and 10 CA cases after COVID-19 infection. To best our knowledge, this is the first report of myelitis with GBS and CA accompanied by multiple autoantibodies. Design/Methods: NA Results: A 60-year-old man with fever and cough was diagnosed with mild COVID-19 infection. Fourteen days later from the onset, he developed gait disturbance and fell frequently. On hospitalization, he exhibited fever, hypoxemia, mild consciousness disturbance, flaccid paraplegia, mild numbness and severe deep sensory disturbance in the lower limbs, bladder and bowel disturbance, mild muscle weakness in the fingers, myoclonus in the extremities, and CA. The PCR of COVID-19 was negative. Blood investigations showed elevated inflammatory markers with dehydration, rhabdomyolysis, and hypercoagulation. Cerebrospinal fluid (CSF) analysis presented mild pleocytosis and elevated protein without anti-COVID-19 antibodies. Contrast-enhanced CT showed massive pulmonary embolisms and deep venous thromboses. Brain SPECT showed cerebellar hypoperfusion despite no abnormalities in brain MRI. Spine MRI revealed longitudinal hyperintense lesions mainly in the dorsal white matter, compatible with myelitis. Additional investigations of autoantibodies realized anti-GM3, TPI, GluR, and NMDAR IgG antibodies in serum, and anti-GluR and NMDAR IgG antibodies with increased granzyme B in CSF. Treatments of corticosteroid and intravenous immunoglobulin resulted in complete recovery to consciousness disturbance, muscle weakness of fingers, myoclonus, and CA, while paraparesis with deep sensory and bladder and bowel disturbance remained. Conclusions: We highlight the possibility of the coexistence of several post-infectious autoimmune neurological complications in patients of COVID-19. It is important to search autoantibodies carefully corresponding to clinical manifestations for appropriate treatments and understanding of pathophysiology.

8.
Neurology ; 98(18 SUPPL), 2022.
Article in English | EMBASE | ID: covidwho-1925420

ABSTRACT

Objective: To describe the occurrence of acute cerebellar ataxia after COVID-19 infection in a 5- year-old boy. Background: Neurologic manifestations can occur in many adult patients with COVID-19 but are less frequently described in the literature than the respiratory or inflammatory effects of the disease. There are even fewer reports of the neurologic manifestations of the disease in children. Design/Methods: A 5-year-old boy with type I diabetes mellitus was exposed to a COVID-19 positive classmate in school. He tested positive for the SARS-CoV-2 virus and developed mild symptoms including rhinorrhea and decreased energy. Eight days later he developed acute ataxia, double vision, tremor and dysmetria. He was admitted to the hospital for further evaluation. Results: He had hyperglycemia at presentation, however labs were not consistent with diabetic ketoacidosis. Nasopharyngeal swab for SARS-CoV-2 was positive by polymerase chain reaction, and SARS-CoV-2 IgG nucleocapsid antibody testing was positive in serum. Cerebrospinal fluid showed white blood cells 8 cells/uL, red blood cells 0 cells/uL, protein 20 mg/dL and glucose 110 mg/dL. Other infectious testing in the CSF was negative including CSF testing for SARS-CoV-2. Brain magnetic resonance imaging with and without contrast was normal. The patient was treated with supportive care and discharged home after 4 days. Symptoms gradually improved and resolved at 2 month follow up. Conclusions: Acute cerebellar ataxia can be seen in children, often following a viral infection. Rare reports have described acute ataxia in adults recovering from COVID-19. Only one case report has previously described acute cerebellar ataxia in a pediatric patient of 13 years (Tomar et al 2021). Providers should be aware of acute cerebellar ataxia as a possible sequela in pediatric patients recovering from COVID-19.

9.
Curr Neurol Neurosci Rep ; 22(8): 499-513, 2022 08.
Article in English | MEDLINE | ID: covidwho-1889030

ABSTRACT

PURPOSE OF REVIEW: To discuss the neurological complications and pathophysiology of organ damage following malaria infection. RECENT FINDINGS: The principal advancement made in malaria research has been a better understanding of the pathogenesis of cerebral malaria (CM), the most dreaded neurological complication generally caused by Plasmodium falciparum infection. However, no definitive treatment has yet been evolved other than the use of antimalarial drugs and supportive care. The development of severe cerebral edema in CM results from two distinct pathophysiologic mechanisms. First, the development of "sticky" red blood cells (RBCs) leads to cytoadherence, where red blood cells (RBCs) get stuck to the endothelial walls and between themselves, resulting in clogging of the brain microvasculature with resultant hypoxemia and cerebral edema. In addition, the P. falciparum-infected erythrocyte membrane protein 1 (PfEMP1) molecules protrude from the raised knob structures on the RBCs walls and are in themselves made of a combination of human and parasite proteins in a tight complex. Antibodies to surfins, rifins, and stevors from the parasite are also located in the RBC membrane. On the human microvascular side, a range of molecules involved in host-parasite interactions, including CD36 and intracellular adhesion molecule 1, is activated during interaction with other molecules such as endothelial protein C receptor and thrombospondin. As a result, an inflammatory response occurs with the dysregulated release of cytokines (TNF, interleukins 1 and 10) which damage the blood-brain barrier (BBB), causing plasma leakage and brain edema. This second mechanism of CNS injury often involves multiple organs in adult patients in endemic areas but remains localized only to the central nervous system (CNS) among African children. Neurological sequelae may follow both P. falciparum and P. vivax infections. The major brain pathology of CM is brain edema with diffuse brain swelling resulting from the combined effects of reduced perfusion and hypoxemia of cerebral neurons due to blockage of the microvasculature by parasitized RBCs as well as the neurotoxic effect of released cytokines from a hyper-acute immune host reaction. A plethora of additional neurological manifestations have been associated with malaria, including posterior reversible encephalopathy syndrome (PRES), reversible cerebral vasoconstriction syndrome (RCVS), malarial retinopathy, post-malarial neurological syndrome (PMNS), acute disseminated encephalomyelitis (ADEM), Guillain-Barré syndrome (GBS), and cerebellar ataxia. Lastly, the impact of the COVID-19 pandemic on worldwide malaria control programs and the possible threat from co-infections is briefly discussed.


Subject(s)
Brain Edema , COVID-19 , Malaria, Cerebral , Malaria, Falciparum , Posterior Leukoencephalopathy Syndrome , Adult , Child , Cytokines , Humans , Hypoxia , Malaria, Cerebral/complications , Malaria, Cerebral/parasitology , Malaria, Falciparum/complications , Malaria, Falciparum/parasitology , Pandemics , Plasmodium falciparum/physiology
10.
Italian Journal of Medicine ; 15(3):40, 2021.
Article in English | EMBASE | ID: covidwho-1567520

ABSTRACT

Background: Neurological manifestations in SARS-CoV-2 are heterogeneous, ranging from mild symptoms such as anosmia and hypogeusia/ ageusia to more severe pictures such as stroke, seizures, ataxia, polyradiculoneuropathy, encephalopathy and movement disorders. Between the neurologic disorders, ataxia is a relatively uncommon manifestation of CNS involvement by SARS-CoV-2. Description of the case: A patient with 78 years of age and other very good general conditions, was admitted to the emergency room after the onset of ataxia associated with the ideomotor slowdown. He had no active disease until the beginning of ataxia, and occasionally he exercises very actively. He had as a single episode of fever (39 C). He performed nose pharyngeal swab, resulted positive. The value of his blood gas analysis was always acceptable, but he had a mild reduction of SpO2 during the walking test. During the hospitalisation, imaging included a chest computed tomography (CT) that showed pneumonia. A brain CT showed a mild hypodensity of the cerebral white matter as chronic cerebrovascular disease. He also performed a neurological consultation. Physicians chose to administer dexamethasone 6 mg iv and enoxaparin sodium 60 mg per Kg of weight subcutaneously, antibiotic therapy and a low flow of oxygen therapy. Conclusions: The particularity of this case is the presence of ataxia as a single symptom of CoViD disease in an elderly patient with SARS-CoV-2 pneumonia. Typical presentations of CoViD-19 generally included symptoms such as fever, dry cough and dyspnoea;in elderly has been often reported the absence of these symptoms.

11.
Pract Neurol ; 21(6): 466-467, 2021 Dec.
Article in English | MEDLINE | ID: covidwho-1526526
12.
Pract Neurol ; 21(6): 554, 2021 Dec.
Article in English | MEDLINE | ID: covidwho-1526525

Subject(s)
Cerebellar Ataxia , Masks , Gait , Humans
13.
Childs Nerv Syst ; 37(7): 2305-2312, 2021 07.
Article in English | MEDLINE | ID: covidwho-1384405

ABSTRACT

PURPOSE: To describe the temporal association of specific acute neurological symptoms in pediatric patients with confirmed SARS-CoV-2 infection between May and August 2020. METHODS: We performed a recollection of all the clinical and laboratory data of patients having acute neurological symptoms temporally associated with SARS-CoV-2 infection at a third-level referral hospital in Mexico City (Instituto Nacional de Pediatría). Patients in an age group of 0-17 years with acute neurological signs (including ascending weakness with areflexia, diminished visual acuity, encephalopathy, ataxia, stroke, or weakness with plasma creatinine kinase (CK) elevation) were evaluated. RESULTS: Out of 23 patients with neurological manifestations, 10 (43%) had a confirmed SARS-CoV-2 infection. Among the infected patients, 5 (50%) were males aged 2-16 years old (median age 11.8 years old). Four (40%) patients confirmed a close contact with a relative positive for SARS-CoV-2, while 6 (60%) cases had a history of SARS-CoV-2-related symptoms over the previous 2 weeks. The following diagnoses were established: 3 cases of GBS, 2 of ON, 2 of AIS, one of myositis with rhabdomyolysis, one ACA, and one of anti-NMDA-R encephalitis. CONCLUSIONS: Neurological manifestations temporally associated with SARS-CoV-2 infection were noticed in the pediatric population even without respiratory symptoms. In this study, 2 of 6 symptomatic patients had mild respiratory symptoms and 4 had unspecific symptoms. During this pandemic, SARS-CoV-2 infection should be considered as etiology in patients with acute neurological symptoms, with or without previous respiratory manifestations, particularly in teenagers.


Subject(s)
COVID-19 , Stroke , Adolescent , Child , Child, Preschool , Humans , Infant , Infant, Newborn , Male , Mexico/epidemiology , Pandemics , SARS-CoV-2
14.
Nutrients ; 13(4)2021 Apr 20.
Article in English | MEDLINE | ID: covidwho-1325744

ABSTRACT

Stiff person syndrome (SPS) is a rare autoimmune disease characterised by axial stiffness and episodic painful spasms. It is associated with additional autoimmune diseases and cerebellar ataxia. Most patients with SPS have high levels of glutamic acid decarboxylase (GAD) antibodies. The aetiology of SPS remains unclear but autoimmunity is thought to play a major part. We have previously demonstrated overlap between anti-GAD ataxia and gluten sensitivity. We have also demonstrated the beneficial effect of a gluten-free diet (GFD) in patients with anti-GAD ataxia. Here, we describe our experience in the management of 20 patients with SPS. The mean age at symptom onset was 52 years. Additional autoimmune diseases were seen in 15/20. Nineteen of the 20 patients had serological evidence of gluten sensitivity and 6 had coeliac disease. Fourteen of the 15 patients who had brain imaging had evidence of cerebellar involvement. Twelve patients improved on GFD and in seven GFD alone was the only treatment required long term. Twelve patients had immunosuppression but only three remained on such medication. Gluten sensitivity plays an important part in the pathogenesis of SPS and GFD is an effective therapeutic intervention.


Subject(s)
Food Intolerance/complications , Glutens/adverse effects , Stiff-Person Syndrome/complications , Adult , Aged , Female , Food Intolerance/diagnostic imaging , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Stiff-Person Syndrome/diagnostic imaging
15.
Medicina (Kaunas) ; 57(4)2021 Apr 01.
Article in English | MEDLINE | ID: covidwho-1241289

ABSTRACT

To date, there is no curable treatment option for non-hereditary degenerative cerebellar ataxia. Here we report the case of a patient with sporadic adult-onset ataxia (SAOA) who underwent allogeneic bone marrow-derived mesenchymal stem cell (MSC) therapy via the intrathecal route. A 60-year-old male patient visited our clinic complaining of progressive gait disturbance that commenced two years ago. Upon neurologic examination, the patient exhibited limb dysmetria and gait ataxia. Brain magnetic resonance imaging (MRI) revealed cerebellar atrophy whereas the autonomic function test was normal. The patient was diagnosed with SAOA. The medications that were initially prescribed had no significant effects on the course of this disease and the symptoms deteriorated progressively. At the age of 64, the patient was treated with allogeneic bone marrow-derived MSC therapy. The subsequent K-SARA (Korean version of the Scale for the Assessment and Rating of Ataxia) scores demonstrated a distinct improvement up until 10 months post-administration. No adverse events were reported. The improved post-treatment K-SARA scores may suggest that the MSC therapy can have a neuroprotective effect and that stem cell therapy may serve as a potential therapeutic option for degenerative cerebellar ataxia.


Subject(s)
Cerebellar Ataxia , Hematopoietic Stem Cell Transplantation , Mesenchymal Stem Cell Transplantation , Mesenchymal Stem Cells , Adult , Bone Marrow , Cerebellar Ataxia/therapy , Humans , Male , Middle Aged
16.
BMC Infect Dis ; 21(1): 298, 2021 Mar 24.
Article in English | MEDLINE | ID: covidwho-1150393

ABSTRACT

BACKGROUND: Severe acute respiratory syndrome virus 2 (SARS-CoV-2) is spreading globally and causes most frequently fever and respiratory symptoms, i.e. Coronavirus disease 2019 (COVID-19), however, distinct neurological syndromes associated with SARS-CoV-2 infection have been described. Among SARS-CoV-2-infections-associated neurological symptoms fatigue, headache, dizziness, impaired consciousness and anosmia/ageusia are most frequent, but less frequent neurological deficits such as seizures, Guillain-Barré syndrome or ataxia may also occur. CASE PRESENTATION: Herein we present a case of a 62-year-old man who developed a subacute cerebellar syndrome with limb-, truncal- and gait ataxia and scanning speech 1 day after clinical resolution of symptomatic SARS-CoV-2 infection of the upper airways. Apart from ataxia, there were no signs indicative of opsoclonus myoclonus ataxia syndrome or Miller Fisher syndrome. Cerebral magnetic resonance imaging showed mild cerebellar atrophy. SARS-CoV-2 infection of the cerebellum was excluded by normal cerebrospinal fluid cell counts and, most importantly, absence of SARS-CoV-2 RNA or intrathecal SARS-CoV-2-specific antibody production. Other causes of ataxia such as other viral infections, other autoimmune and/or paraneoplastic diseases or intoxication were ruled out. The neurological deficits improved rapidly after high-dose methylprednisolone therapy. CONCLUSIONS: The laboratory and clinical findings as well as the marked improvement after high-dose methylprednisolone therapy suggest a post-infectious, immune-mediated cause of ataxia. This report should make clinicians aware to consider SARS-CoV-2 infection as a potential cause of post-infectious neurological deficits with an atypical clinical presentation and to consider high-dose corticosteroid treatment in case that a post-infectious immune-mediated mechanism is assumed.


Subject(s)
COVID-19/complications , Cerebellar Ataxia/complications , Cerebrum/diagnostic imaging , Humans , Male , Middle Aged , RNA, Viral
17.
J Emerg Med ; 60(1): 73-76, 2021 01.
Article in English | MEDLINE | ID: covidwho-1065315

ABSTRACT

BACKGROUND: The outbreak of coronavirus disease 2019 (COVID-19) has been widely reported to cause symptoms such as fever, cough, sore throat, fatigue, and shortness of breath. Neurologic complications have not been widely reported without associated respiratory symptoms. These neurologic manifestations have been found mostly in the elderly. There has been no report of ataxia or COVID-19 cerebellitis in the young adult population without associated respiratory symptoms. CASE REPORT: Here we report the case of a 30-year-old patient who presented with isolated cerebellar symptoms and was diagnosed with COVID-19 cerebellitis. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: It is important for emergency physicians to know that COVID-19 can have many clinical manifestations and to have a high level of suspicion with acute neurologic symptoms.


Subject(s)
COVID-19/complications , Cerebellar Ataxia/etiology , Adult , COVID-19/virology , Cerebellar Ataxia/diagnostic imaging , Cerebellar Ataxia/physiopathology , Humans , Male
18.
Mov Disord Clin Pract ; 7(8): 974-976, 2020 Nov.
Article in English | MEDLINE | ID: covidwho-808460
19.
J Neurol ; 268(7): 2343-2345, 2021 Jul.
Article in English | MEDLINE | ID: covidwho-777819

ABSTRACT

The coronavirus disease 2019 (COVID-19) crisis confronted us, like many researchers worldwide, with an unforeseen challenge during the final stages of a randomized controlled trial involving ataxia patients. Institutional guidelines suddenly no longer allowed regular follow-up visits to take place, impeding the clinical evaluation of long-term outcomes. Here, we discuss the various scenarios that we considered in response to these imposed restrictions and share our experience of home video recording by dedicated, extensively instructed family members. Albeit somewhat unconventional at first glance, this last resort strategy enabled us to reliably assess the study's primary endpoint at the predefined point in time and hopefully encourages researchers in other ongoing ataxia trials to continue their activities. Remote assessments of ataxia severity may serve as a reasonable substitute in interventional trials beyond the current exceptional situation generated by the COVID-19 pandemic, but will require further investigation.


Subject(s)
Ataxia/therapy , COVID-19 , Pandemics , Randomized Controlled Trials as Topic , Humans
20.
Cerebellum ; 19(4): 562-568, 2020 Aug.
Article in English | MEDLINE | ID: covidwho-267192

ABSTRACT

The current worldwide severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic that causes coronavirus disease 2019 (COVID-19) has brought some medical systems to the brink of collapse. This crisis is also negatively impacting the care of patients with non-COVID-19 conditions, including those with cerebellar ataxia (CA). Older patients with CA and those with immune-mediated ataxias on immunosuppressive medication are potentially at high risk of developing serious complications of the infection, although it is also possible that immunosuppressive agents may provide a defense against cytokine storm. This has implications for even greater attention to preventing contracting the disease through physical distancing and/or isolation. The CA patient population is also at higher risk because of the neurological complexities of their underlying disorder and the comorbid medical illnesses that often accompany the genetic ataxias. As the disruption of social patterns and healthcare delivery in response to the crisis continues, interruption of rehabilitation, speech and language therapy, and face-to-face consultations threatens to have a negative impact on the course and well-being of CA patients. Mental and physical health is also potentially at greater risk because the prevailing uncertainty and anxiety may be superimposed upon cerebellum-specific neuropsychological challenges. We identify and review some of the short- and long-term consequences of this global pandemic for the community of ataxia patients and their families and for the clinical and academic neurologists/ataxiologists caring for these patients. This includes the recognition that telemedicine has emerged as a principle means of caregiver-patient contact and that neurological manifestations of COVID-19 including those specific to cerebellar neurobiology are increasingly recognized and will require close surveillance and monitoring. This COVID-19 Cerebellum Task Force consensus provides some guidance on how we may approach this uncertain time and consider preparing for the new realities we face in CA patient care once this acute crisis has passed.


Subject(s)
Cerebellar Ataxia , Coronavirus Infections , Pandemics , Pneumonia, Viral , Betacoronavirus , COVID-19 , Cerebellar Ataxia/epidemiology , Cerebellar Ataxia/virology , Comorbidity , Coronavirus Infections/epidemiology , Humans , Pneumonia, Viral/epidemiology , Risk Factors , SARS-CoV-2
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