Management of Patients with Cerebellar Ataxia During the COVID-19 Pandemic: Current Concerns and Future Implications.

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.

[Varicella is not always benign]. / La varicelle n'est pas toujours bénigne.

Varicella is a rash illness caused by the varicella-zoster virus. It mainly affects children with usually a benign outcome. However, several complications of variable severity can be observed including bacterial infectious complications and neurological complications. We report two cases of complicated varicella. Case 1: 5 month old baby with no previous pathological history presenting with a rash composed of vesicles and pustules lasting for six days. Symptoms worsened the day before his admission to our Department due to respiratory distress. Case 2: 7-Year old girl admitted to our Department due to simple convulsion. Clinical examination showed generalized varicella scars and cerebellar ataxia. Although varicella is a common, in most cases benign viral disease, several studies have recently reported a recrudescence of complications, which appear to be responsible for 0.2-1.5% of the causes of hospitalization in children with varicella.

Barr humbug: acute cerebellar ataxia due to Epstein-Barr virus.

Epstein-Barr virus (EBV) infection is associated with neurological sequellae, but rarely there is acute cerebellar ataxia (ACA) in an adult. We present a novel case of a 26-year-old man, who presented with ACA. He had normal MRI and CSF analysis. Serum testing confirmed active EBV. A course of oral prednisolone 1 mg/kg for 4 weeks, with a subsequent taper was started. He made a full recovery within 3 weeks of presentation.

HIV Presenting as Cerebellar Ataxia.

Presenting features of HIV has always been a Pandora's box largely due to multisystem affection by the virus and a large array of opportunistic infections. We hereby report an extremely rare case of 40 yr old patient presenting as symmetric, progressive cerebellar ataxia later found to be HIV positive. Thorough knowledge of rare presentations of HIV and a high index of suspicion are necessary for early diagnosis and efficient treatment of HIV.

Varicella Zoster Virus Cerebellitis Without a Rash in an Immunocompetent 85-Year-Old Patient.

BACKGROUND: Reactivation of the varicella zoster virus (VZV) in adults rarely presents with neurological symptoms without a rash. To our knowledge, so far, only 3 additional cases of VZV cerebellitis, which presented without a rash and were proven by cerebrospinal fluid analysis, have been reported in the literature. CASE REPORT: An immunocompetent 85-year-old patient presented with a new-onset tremor. He had no rash, had a normal brain computed tomography and magnetic resonance imaging, and had minimal cerebrospinal fluid findings. Eventually, he was diagnosed as having varicella zoster virus cerebellitis only on the basis of a virological examination. CONCLUSIONS: The manifestation of a new-onset tremor and gait ataxia should raise a suspicion of cerebellitis caused by VZV, even in the absence of cutaneous manifestations or typical imaging findings.

A case of Murray Valley encephalitis in a 2-year-old Australian Stock Horse in south-east Queensland.

CASE REPORT: This report summarises the findings from a case of naturally-occurring Murray Valley encephalitis in a 2-year-old filly presenting with acute onset of depression and weakness. Serum samples tested at the onset of clinical signs were negative for Hendra and Kunjin virus antibodies, but positive for Murray Valley encephalitis virus (MVEV) using IgM-capture ELISA (1 : 300 dilution). A virus neutralisation assay performed 4 weeks later confirmed a titre of 1 : 160. Sera collected in the weeks preceding neurological signs returned a negative titre for MVEV 2 weeks prior followed by a titre of 1:80 in the week prior to illness. Serological surveillance conducted on 67 co-located horses returned a positive titre of 1 : 20 in one in-contact horse. There was no history of clinical disease in that horse. At 3 months after the onset of clinical signs in the index case, the filly continued to show mild facial paresis and hypermetria; the owners elected euthanasia and gave permission for necropsy. Histopathological analysis of the brain showed a mild meningoencephalitis. CONCLUSION: The progression of a naturally-occurring MVEV infection in a horse has been documented in this case.

A Case of Cerebellar Ataxia Associated with HIV Infection.

Cerebellar complications of HIV infection primarily manifested in ataxia, usually arise as the result of cerebellar lesions due to opportunistic infections, vasculitis or neoplastic processes. A 28 year old female known to have HIV infection for last four years, presented to our hospital with progressive unsteadiness in walking, slurring of speech and intention tremors for the last two months. There was no family history of similar complaints, and she was on Anti retroviral treatment for last one and a half years. The results of examination were notable for severe dysarthria, slow saccades, a conspicuous dysmetria and dysdiadokokinesia. She had no cognitive, sensory or motor deficits. MRI revealed diffuse cerebellar atrophy. Extensive laboratory work up failed to disclose a cause for subacute ataxia. Isolated cerebellar degeneration in an HIV patient is rare and should prompt a diagnostic work up.

JC polyomavirus granule cell neuronopathy in a patient treated with rituximab.

IMPORTANCE: Progressive multifocal leukoencephalopathy results from lytic infection of the glia by the JC polyomavirus (JCV); JCV granule cell neuronopathy is caused by infection with a mutated form of JCV, leading to a shift in viral tropism from the glia to cerebellar granule cells. This shift results in a clinical syndrome dominated by progressive cerebellar dysfunction that might elude standard diagnostic workup strategies for ataxia. OBSERVATIONS: We present the case report of a patient receiving long-term rituximab therapy who developed progressive cerebellar ataxia and marked isolated cerebellar degeneration. This syndrome resulted from JCV granule cell neuronopathy associated with a novel JCV mutation. CONCLUSIONS AND RELEVANCE: New onset or worsening of isolated cerebellar ataxia in patients being treated with rituximab or natalizumab warrants early assessment for JCV infection.

Epstein-Barr virus-associated cerebellar ataxia.

Cerebellar ataxia is a common neurological presentation. It can be acute, subacute or chronic. Neurological complications of Epstein-Barr virus (EBV) are well-recognised with a variety of presentations. Acute cerebellar ataxia is a rare, but an established complication. It has been described as the sole manifestation of EBV infection without the systemic features of infectious mononucleosis. The pathophysiology is not clear. The course of the illness may last for a few months with a benign outcome, though serious complications can happen. We present a case of a 38-year-old man who presented with an acute cerebellar ataxia owing to EBV infection, along with a review of the literature.

[Clinical significance and the first identification of human parechoviruses in Hungary]. / A humán parechovírusok klinikai jelentosége és elso hazai azonosítása.

UNLABELLED: Human parechoviruses (HPeV) belonging to the family Picornaviridae are widespread enteric pathogens and are associated with various clinical syndromes in human. At present, 16 HPeV genotypes (HPeV1-16) are known. There is no report on the detection of HPeVs in Central Europe. AIMS: The aim of the retrospective study was to detect and characterize HPeVs using molecular methods in cell cultures with "enterovirus-like" cytophatic effect (CPE) archived between 1990 and 2004, in two virology laboratories, in Hungary. MATERIALS AND METHODS: In Laboratory I, fecal samples from children with symptoms of gastroenteritis under the age of 10 years were cultured as a previous routine diagnostic laboratory protocol for "enterovirus". Cell cultures indicating CPE were archived between 1990 and 2000. In Laboratory II, 2 fecal samples, a liquor and a nasopharyngeal aspirate were re-tested which contained an "enterovirus-like" virus in cell cultures and were positive by HPeV1 neutralization immunosera between 2000 and 2004. Specimens were tested retrospectively for HPeV by reverse transcription-PCR (RT-PCR) method using 5'UTR conserved primers. Specific primers were designed to determine the HPeV structural region (VP0-VP3-VP1). RESULTS: 9 of the 66 archived samples (9.1%) from Laboratory I and all the 4 samples from Laboratory II were found to be HPeV-positive. 10 samples were identified as HPeV1, 2 were HPeV4 and 1 could not be determined. 3 HPeV1 clusters were identified in Laboratory I according to the isolation date originated from years 1990/1991, 1992/1995 and 1998. HPeV1 was detected in clinical syndromes: gastroenteritis (in a 24-years-old adult), recurrent stomatitis aphtosa (in a 42-years-old adult), encephalitis and ataxia cerebellaris acuta in infants and children in Laboratory II. CONCLUSIONS: This is the first detection of HPeVs in Central Europe. Detection and genetic characterization of HPeV in available historical samples infected with previously unidentifiable agents with "enterovirus-like" cytopathogenic effect may help to understand the clinical importance and spectrum of the infections and the genetic diversity and evolution of these viruses.

Severe ataxia as a complication of human parvovirus B19 acute encephalitis in a child.

Human parvovirus B19 generally causes erythema infectiosum in childhood, but it can be associated with unusual findings, particularly in immunocompromised patients. This is a report about an immunocompetent 4-year-old female child affected with acute encephalitis by parvovirus B19, documented by polymerase chain reaction performed on cerebrospinal fluid, who was treated with intravenous immunoglobulins and dexamethasone and who developed a cerebellar syndrome with ataxia, dysmetria, and dysarthria. To the best of the authors' knowledge, this may be the first report of human parvovirus B19 encephalitis complicated by severe ataxia in childhood.

An increasing incidence of chickenpox central nervous system complications in children: what's happening in Tuscany?

BACKGROUND: The most frequent noncutaneous site of involvement of chickenpox is the central nervous system (CNS) and complications include cerebellar ataxia, encephalitis, and meningitis. OBJECTIVES: We have recently observed an unusually high number of children with chickenpox CNS complications in our university children's hospital. A study to evaluate the incidence of these complications over time in children living in Tuscany was carried out. STUDY DESIGN: We evaluated all cases of chickenpox and chickenpox complications leading to hospitalization in children aged 1 month-14 years reported to the Tuscany public health centre between 1997 and 2004. The International Classification of Disease Ninth Revision-CM hospital discharge diagnostic codes and medical records were used. RESULTS: The incidence (95% confidence interval) of CNS complications/1000 chickenpox cases was stable between 1997 and 2001 [1997: 0.80 (0.29-1.74); 1998: 0.73 (0.29-1.50); 1999: 0.67 (0.25-1.46); 2000: 0.56 (0.15-1.44); 2001: 0.59 (0.16-1.50)] but increased significantly (chi(2) for trend: 9.401; p=.0021) in 2002 [1.56 (0.83-2.66)], in 2003 [1.73 (0.95-2.90)] and in 2004 [1.51 (0.74-2.27)]. Non-CNS complications remained stable over time. CONCLUSIONS: Possible factors biasing the result were taken into account. Reasons of increased CNS complications remain unknown, but the possible emergence of a particularly neurotropic strain of varicella-zoster virus should be further investigated.