Transient Generalized Chorea in Influenza A Encephalopathy.

Background: Influenza A infections are a rare cause of movement disorders. Previously described patients have suffered from acute-onset myoclonus and/or dystonia or post-viral parkinsonism. Case Report: We present the case of a 74-year-old female patient with transient generalized chorea due to influenza A-mediated encephalopathy. Discussion: We discuss whether the clinical presentation and the magnetic resonance imaging changes may be attributable to cytokine-mediated encephalopathy or to direct cytotoxic effects of the virus. Additionally, we would like to make clinicians aware of this clinical sign in the context of viral encephalopathy.

[Post-herpes simplex encephalitis chorea: Viral replication or immunological mechanism?]. / Chorée aiguë après encéphalite herpétique : réplication virale ou mécanisme immunologique ?

INTRODUCTION: Herpes simplex encephalitis is a severe neurological condition, whose outcome is improved if treated early with acyclovir. Post-herpes simplex encephalitis with acute chorea has rarely been reported. CASE REPORT: We report on two observations of children presenting with post-herpes simplex encephalitis with acute chorea, related to two different pathophysiological mechanisms. The first one is an 11-month-old girl developing relapsing herpes simplex encephalitis with chorea due to resumption of viral replication. The second one is a 2-year-old boy with relapsing post-herpes simplex encephalitis acute chorea caused by an immunoinflammatory mechanism. We discuss the different neurological presentations of herpetic relapses, notably those presenting with movement disorders, as well as their clinical, paraclinical, physiopathological, and therapeutic aspects. CONCLUSION: Post-herpes simplex encephalitis with acute chorea may involve two mechanisms: resumption of viral replication or an immunoinflammatory mechanism. Treatment of post-herpes simplex encephalitis with acute chorea depends on the underlying mechanism, while prevention is based on antiviral treatment of herpes simplex encephalitis with acyclovir at the dose of 20mg/kg/8h for 21 days.

Canine paroxysmal movement disorders.

Paroxysmal dyskinesias are episodic movement disorders characterized by muscle hypertonicity that can produce involuntary movements. Signs emanate from the central nervous system; consciousness is not impaired, ictal electroencephalography is normal, and there are no autonomic signs, distinguishing them from seizure disorders. In humans they are classified into 3 groups, each responding to different therapies. A mutation in the gene for brevican (BCAN) has been identified as the cause of Episodic Falling in Cavalier King Charles spaniels. Further elucidation of the genetic causes will enhance our ability to identify and treat these canine diseases.

Unusual trigeminal autonomic pain heralding hemichorea due to zoster sine Herpete vasculopathy.

BACKGROUND: Varicella zoster virus primary infection is responsible for chickenpox, whereas secondary infection or reactivation can lead to a variety of clinical scenarios. If latent infection is established in trigeminal ganglion, the reactivation can determine viral migration to cerebral arteries, which causes a cerebral vasculopathy and subsequently an ischemic stroke. PATIENTS: Here we report on a child experiencing recurrent episodes of headache mimicking a trigeminal autonomic cephalalgia, in the absence of any skin rash, which were followed by the occurrence of an ipsilateral hemiparesis associated with a choreic movement disorder a month later. RESULTS: Magnetic resonance angiography showed evidence of a right-sided infarction of basal ganglia and anterior limb of the internal capsule, corresponding to the vascular territory of the recurrent artery of Heubner, as a consequence of a focal varicella zoster virus arteriopathy. CONCLUSIONS: We suggest that the recognition of this prodromal manifestation, which can be interpreted as a zoster sine herpete, could provide clinicians an extremely useful time window to start promptly with a prophylactic treatment.

Chorea and developmental regression associated with human herpes virus-6 encephalitis.

We report a 14-month old child with multiple episodes of febrile status epilepticus, followed by chorea and developmental regression, caused by human herpes virus-6 encephalitis. Chorea has been described as a complication of relapsing herpes simplex virus I infection, but not as a manifestation of human herpes virus-6 infection. It is uncertain whether the chorea was an autoimmune phenomenon or a direct effect of the virus. The child was treated with levetiracetam, intravenous immunoglobulin, and foscarnet. The seizures and chorea resolved with treatment, but developmental regression, with loss of language skills, persisted 6 months after the illness. This child illustrates a new clinical presentation of human herpes virus-6 encephalitis, adds to the spectrum of disorders caused by this virus, and strengthens the case for routine identification of specific viral agents in all cases of childhood viral infections with central nervous system symptoms to determine optimal treatment and prognosis.

The role of viral agents in aetiopathogenesis of acute rheumatic fever.

The reason why abnormal immune response exists in acute rheumatic fever is not exactly explained. The influence of co-pathogens like certain viruses were mentioned regarding the initiation of the immunological reaction in acute rheumatic fever patients by several authors since 1970. This study was designed to find the role or effect of some viral infections in the development of rheumatic fever. In this study, 47 cases with acute rheumatic fever (acute rheumatic arthritis, acute rheumatic carditis, and chorea), 20 cases with chronic rheumatic fever, 20 cases with streptococcal pharyngitis, and 20 healthy age- and gender-matched control cases were involved. Serological and molecular tests were made including hepatitis B virus, hepatitis C virus, rubella virus, herpes simplex virus (HSV group 1), and Epstein-Barr virus (EBV). HBsAg, rubella IgM and EBV IgM positivity were not seen in any of patients with rheumatic fever. Although antiHBs seropositivity was higher in the control group, it was not statistically significant (p > 0.05). There was no difference in rubella IgG, HSV IgM seropositivity, either (p > 0.05). EBV DNA was searched by the polymerase chain reaction technique; due to the latent nature of the virus, no significant difference was found between the control group and the other groups (p > 0.05). In this study, no positive correlation could be found to support the synergism theories regarding the streptoccocus infection and viral infections in the development of acute rheumatic fever. Only EBV DNA positivity was found in all acute rheumatic fever cases but not in the control group may lead to further studies with larger series of patients.

Childhood chorea-encephalopathy associated with recent parvovirus B19 infection in two Jamaican children.

This case report highlights the course of two healthy unrelated children with an encephalopathy characterised by dyskinesia, seizures, hemiparesis and behavioural change associated with recent human parvovirus B19 infection. The cases are compared with a previously described case of childhood chorea encephalopathy associated with human parvovirus B19 infection.

Childhood chorea-encephalopathy associated with human parvovirus B19 infection.

An 8-year-old female presented with a distinct clinical course characterized by an acute self-limiting chorea-encephalopathy with cerebrospinal fluid (CSF)-specific oligoclonal bands. During the clinical course, genomic human parvovirus B19 DNA was detected in her serum and CSF. It was concluded that this patient represents the first published case of childhood chorea-encephalopathy associated with, and probably caused by, human parvovirus B19 infection.

Chorea: non-genetic causes.

PURPOSE OF REVIEW: The aim of this article is to review the literature on the non-genetic causes of chorea. The differential diagnosis of the large number of causes of sporadic chorea is often a challenging task. Interest has also been growing in the possibility that the mechanism responsible for Sydenham's chorea plays a role in the pathogeneis of other neuropsychiatric disorders. RECENT FINDINGS: Stroke is the main cause of sporadic chorea. Sydenham's chorea shares clinical features with tic disorders, such as obsessive-compulsive disorder and attention deficit hyperactivity disorder. However, there are unequivocal differences between Sydenham's chorea and Tourette's syndrome. There is initial evidence suggesting the beneficial effect of immunosuppression in Sydenham's chorea. Other autoimmune causes of chorea include systemic lupus erythematosus as well as paraneoplastic syndromes. The growing list of drugs associated with chorea include lamotrigine, methadone and lithium. Among infectious agents, HIV is the leading reported cause of chorea. SUMMARY: Patients with sporadic chorea require a thorough work up because numerous causes can lead to this condition. It remains unconfirmed whether the pathogenic mechanisms of Sydenham's chorea are responsible for other conditions such as isolated obsessive-compulsive disorder or Tourette's syndrome. Drugs and infectious agents, especially HIV, are often implicated in the causes of chorea.

Chorea in patients with AIDS.

OBJECTIVE: To describe differing etiologies and possible anatomoclinical correlates of choreic movements in a series of AIDS patients. METHODS: We analyzed the clinical records and neuroimaging data of 5 consecutive AIDS patients who developed choreic movements at our center from January, 1994 to December, 1996. RESULTS: There were 2 cases of focal choreic dyskinesias, 1 of right hemichorea, and 2 of generalized chorea. Onset was acute and febrile in 1 case, and subacute in the other 4. In 1 patient the chorea was the AIDS onset symptom; in another choreic movements were the first neurological symptom following AIDS diagnosis; in 2 patients AIDS had a neurological onset other than chorea; and in the fifth patient buccofacial dyskinesias appeared following the development of bacterial encephalitis. CONCLUSION: Chorea was associated with cerebral toxoplasmosis in 2 patients, progressive multifocal leukoencephalopathy in 1, subacute HIV encephalopathy in another, and was probably iatrogenic in the last. Chorea is not unusual in AIDS, however the causes are variable and careful neuroradiological and clinical evaluation is required to identify them. AIDS-related disease should be considered in young patients presenting with chorea without a family history of movement disorders.