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Related Concept Videos

Encephalitis l: Introduction01:19

Encephalitis l: Introduction

Encephalitis is inflammation of the brain parenchyma, most often due to infections or autoimmune processes. It presents with neuropsychiatric features such as fever, altered mental status, behavioral changes, cognitive dysfunction, seizures, focal deficits, and sometimes autonomic instability. In some cases, the meninges are also involved, resulting in meningoencephalitis.Infectious CausesInfectious encephalitis is most commonly viral but can also result from bacterial, fungal, or parasitic...
Encephalitis ll: Pathophysiology01:26

Encephalitis ll: Pathophysiology

Encephalitis is inflammation of the brain parenchyma caused by direct viral invasion or immune-mediated mechanisms triggered by infections or tumors. Both processes lead to neuronal injury, disrupted neurotransmission, and diverse neurological symptoms, often with overlapping clinical and pathological features.Autoimmune EncephalitisIn autoimmune encephalitis, antibodies target neuronal antigens on cell surfaces, synapses, or within neurons. A key example is anti-NMDAR encephalitis, which can...
Arboviral Encephalitis01:25

Arboviral Encephalitis

Arboviral encephalitis refers to brain inflammation caused by arthropod-borne viruses, particularly those transmitted through mosquito vectors. Among these, West Nile virus (WNV), a member of the Flaviviridae family, is a significant public health concern. WNV is an enveloped, positive-sense, single-stranded RNA virus. Human infection typically begins when an infected mosquito introduces the virus into the dermis during feeding. The primary transmission cycle involves birds as amplifying hosts...
Bacterial Meningitis I: Introduction01:22

Bacterial Meningitis I: Introduction

Bacterial meningitis is a severe, life-threatening inflammation of the meninges, particularly the pia mater and arachnoid mater, affecting the subarachnoid space, ventricles, and cerebrospinal fluid (CSF). If untreated, it can lead to significant neurological complications or death.Causative AgentsCommon pathogens vary with age and immune status. In adults, major organisms include Streptococcus pneumoniae, Neisseria meningitidis, and Haemophilus influenzae. Streptococcus agalactiae (group B...
Viral Meningitis01:18

Viral Meningitis

Viral meningitis is the most common form of meningitis and is often referred to as aseptic meningitis to indicate the absence of bacterial involvement. It is generally milder than bacterial meningitis, with symptoms including fever, headache, stiff neck, drowsiness, nausea, photophobia, and vomiting. Rarely, more severe manifestations or death may occur. Common causative agents include enteroviruses, particularly coxsackie A and B viruses and echoviruses, all members of the Enterovirus genus...
Bacterial Meningitis II: Pathophysiology01:26

Bacterial Meningitis II: Pathophysiology

Bacterial meningitis typically begins when pathogens such as Neisseria meningitidis and Streptococcus pneumoniae colonize the nasopharynx and invade the bloodstream. This process is facilitated by bacterial virulence factors, such as polysaccharide capsules, which resist phagocytosis and complement-mediated killing. Less commonly, bacteria reach the central nervous system via contiguous spread from infections like otitis media or sinusitis, through congenital or acquired dural defects, or...

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Rhomb- and bickerstaff encephalitis: two clinical phenotypes?

Rebecca C Gologorsky1, Jerome A Barakos, Farhad Sahebkar

  • 1Division of Pediatric Neurology, California Pacific Medical Center, San Francisco, California, USA. rgologorsky@gmail.com

Pediatric Neurology
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Summary

This study details two unusual pediatric brainstem encephalitis cases with varying symptoms, highlighting potential deviations from typical neurological disorder presentations.

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Area of Science:

  • Neurology
  • Pediatric Neurology
  • Neuroimmunology

Background:

  • Brainstem encephalitis is a rare neurological condition affecting the brainstem.
  • Understanding its varied clinical presentations is crucial for accurate diagnosis and management.
  • The Miller-Fisher syndrome/Bickerstaff brainstem encephalitis/Guillain-Barré syndrome spectrum represents a known group of related disorders.

Observation:

  • Two young boys presented with acute febrile illness, progressive encephalopathy, and cerebrospinal fluid pleocytosis.
  • Case one exhibited ophthalmoplegia and ataxia, consistent with some prior reports.
  • Case two presented similarly but lacked ophthalmoplegia and ataxia, indicating phenotypic variability.

Findings:

  • Magnetic resonance imaging (MRI) revealed distinct patterns of lesion localization between the two patients.
  • Neuroanatomic differences correlated with the observed variations in clinical symptoms.
  • The clinical features in these cases deviated from the classical presentations within the Miller-Fisher syndrome/Bickerstaff brainstem encephalitis/Guillain-Barré syndrome spectrum.

Implications:

  • These cases underscore the importance of considering atypical presentations of brainstem encephalitis in pediatric patients.
  • Further research is needed to elucidate the causes of phenotypic variability in these conditions.
  • Recognizing neuroanatomic correlates of symptom variation can aid in differential diagnosis and patient care.