Choroiditis and choroidal neovascularization in acute disseminated encephalomyelitis

Nikol Panou1, Ivana K Kim, Lucia Sobrin

  • 1*Department of Ophthalmology, Zoodochos Pigi Wing, Amalia Fleming Prefectoral General Hospital, Athens, Greece †Retina ‡Uveitis Services, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts.

Insights

This case study highlights a patient with bilateral choroidal neovascularization (CNV) secondary to acute disseminated encephalomyelitis (ADEM)-associated choroiditis. Immunomodulatory therapy successfully prevented CNV recurrence, suggesting a role for systemic inflammation.

Area of Science:

  • Ophthalmology
  • Neurology
  • Immunology

Background:

  • Acute disseminated encephalomyelitis (ADEM) is a rare, immune-mediated demyelinating disease.
  • Choroiditis and secondary choroidal neovascularization (CNV) are uncommon complications of ADEM.
  • Recurrent CNV poses a significant challenge in managing ADEM-associated ocular manifestations.

Purpose of the Study:

  • To report a unique case of bilateral CNV secondary to ADEM-associated choroiditis.
  • To evaluate the efficacy of immunomodulatory therapy in preventing CNV recurrence.
  • To discuss the role of systemic inflammation in the pathogenesis of ADEM-related CNV.

Main Methods:

  • A 57-year-old male patient with a history of ADEM was diagnosed with bilateral CNV 6 years post-neurologic event.
  • Initial treatment involved intravitreal bevacizumab and oral prednisone.
  • Recurrence upon steroid tapering led to the initiation of mycophenolate mofetil as steroid-sparing immunomodulatory therapy.

Main Results:

  • Intravitreal bevacizumab and oral prednisone initially controlled the CNV.
  • CNV recurred during oral steroid tapering.
  • Mycophenolate mofetil achieved sustained remission of CNV for 1.5 years without further antiangiogenic therapy.

Conclusions:

  • This is the first reported case of choroiditis and secondary CNV associated with ADEM.
  • Systemic immunomodulatory therapy, in conjunction with antiangiogenic agents, should be considered for recurrent CNV in ADEM.
  • The findings support the hypothesis that ongoing inflammation plays a crucial role in the pathogenesis of ADEM-associated CNV.
Abstract

Related Concept Videos

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...
9
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...
5
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...
52
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...
165
Hepatic Encephalopathy01:29

Hepatic Encephalopathy

DefinitionHepatic encephalopathy is a reversible neurologic syndrome that results from advanced liver dysfunction or portosystemic shunting. It leads to disturbances in cognition, behavior, and motor function due to the brain’s exposure to gut-derived toxins that the liver fails to detoxify.EtiologyThis condition develops either in the setting of acute fulminant hepatitis or progressively during chronic liver disease, such as cirrhosis and portal hypertension. Portosystemic...
18
Cerebral Edema ll: Pathophysiology01:22

Cerebral Edema ll: Pathophysiology

Vasogenic edema is a major form of cerebral edema characterized by abnormal accumulation of fluid in the brain’s extracellular space due to disruption of the blood–brain barrier (BBB). The BBB is a specialized structure composed of endothelial cells connected by tight junctions, supported by astrocytic endfeet and a basement membrane. Under normal conditions, it tightly regulates the movement of ions, proteins, and solutes between the bloodstream and brain parenchyma. When this...
2