Central nervous system inflammatory demyelinating disorders of childhood

Mahesh Kamate1, Vivek Chetal, Venkatesh Tonape

  • 1Department of Pediatrics, KLE University's J N Medical College, Belgaum, Karnataka, India.

Insights

Childhood Central Nervous System (CNS) inflammatory demyelinating disorders (CIDD) are increasingly diagnosed. This study analyzed the clinical and radiological profiles and outcomes of pediatric CIDD cases, finding varied presentations and outcomes.

Area of Science:

  • Pediatric Neurology
  • Neuroimmunology
  • Demyelinating Disorders

Background:

  • Childhood Central Nervous System (CNS) inflammatory demyelinating disorders (CIDD) are increasingly recognized.
  • Diagnostic ambiguity and varying prevalence necessitate standardized definitions.
  • Recent consensus definitions aim to improve global study uniformity.

Purpose of the Study:

  • To investigate the clinico-radiological profile of pediatric CIDD.
  • To evaluate treatment outcomes in children diagnosed with CIDD.
  • To apply recent consensus definitions for CIDD diagnosis.

Main Methods:

  • Prospective descriptive study design.
  • Enrollment of pediatric patients with suspected CIDD from July 2007 to August 2008.
  • Analysis of clinical presentation, neuroimaging, laboratory results, treatment, and outcomes.

Main Results:

  • Fifteen pediatric CIDD cases were identified: 11 with acute disseminated encephalomyelitis and 4 with clinically isolated syndrome.
  • Clinical presentations were diverse.
  • Outcomes included complete recovery (8 patients), sequelae (4 patients), and mortality (3 patients). No cases of multiple sclerosis or neuromyelitis optica were observed.

Conclusions:

  • CNS inflammatory demyelinating disorders are prevalent in developing nations, often linked to recurrent infections.
  • The spectrum of CIDD may differ geographically.
  • Magnetic resonance imaging is crucial for diagnosing CIDD.
Abstract

Related Concept Videos

Multiple Sclerosis l: Introduction01:19

Multiple Sclerosis l: Introduction

Multiple sclerosis is a chronic autoimmune disease of the central nervous system (CNS) that affects the brain, spinal cord, and optic nerves. It is an inflammatory demyelinating disorder and a leading cause of neurological disability in young adults.EpidemiologyMS commonly begins between 20 and 40 years of age and is twice as common in women. Its exact cause remains unclear, but genetic susceptibility contributes, with higher risk in first-degree relatives and identical twins. A greater...
Disorders of the Nervous Tissue01:28

Disorders of the Nervous Tissue

Nervous tissue is a vital component of the human body's communication system, enabling us to perceive and respond to stimuli. However, like all other tissues, it is vulnerable to disorders and diseases that can significantly impact our neurological functioning.
Homeostatic Imbalances:
Alzheimer's disease manifests as a gradual decline in memory and cognitive abilities, attributed to the buildup of amyloid plaques and neurofibrillary tangles in the brain.
Parkinson's disease arises from the...
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...
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...
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...