A case of idiopathic encephalomeningocele

Athanasios K Petridis1, Alexandros Doukas1, Hubertus M Mehdorn1

  • 1Department of Neurosurgery, University Hospital of Schleswig-Holstein, Campus Kiel, Germany.

Clinics and Practice
|April 26, 2014
PubMed

Insights

This case study details an adult temporobasal encephalomeningocele, a rare condition typically seen in children. Surgical removal successfully resolved the patient's chronic headaches.

Area of Science:

  • Neurosurgery
  • Neurology
  • Congenital Abnormalities

Background:

  • Encephalomeningocele, a neural tube defect, predominantly affects children, causing facial disfigurement.
  • Adult cases are rare, often presenting with rhinorrhea, but this case highlights an atypical presentation.
  • The etiology is typically a congenital cranial base fusion defect, though not apparent in this patient.

Purpose of the Study:

  • To report a rare case of temporobasal encephalomeningocele in an adult female.
  • To describe the diagnostic process and successful surgical management of this condition in an elderly patient.
  • To highlight the varied clinical presentations of encephalomeningocele in adults.

Main Methods:

  • Diagnostic imaging included brain magnetic resonance imaging (MRI), cranial computed tomography (CT), and MR cisternography.
  • Surgical intervention involved a pterional approach for complete removal of the encephalomeningocele.
  • Clinical assessment of symptoms, particularly headaches and rhinorrhea, was performed pre- and post-operatively.

Main Results:

  • A 72-year-old female presented with chronic headaches, lacking typical symptoms like rhinorrhea.
  • Imaging confirmed a temporobasal encephalomeningocele.
  • Complete surgical excision of the lesion was achieved.
  • The patient's headaches resolved completely following surgery.

Conclusions:

  • Temporobasal encephalomeningocele can occur in adults without apparent congenital causes and may present atypically with headaches as the primary symptom.
  • Advanced imaging techniques are crucial for diagnosing such rare conditions.
  • Surgical intervention offers a definitive treatment, leading to symptom resolution.

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...
15
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...
19
Brain Abscess l: Introduction01:26

Brain Abscess l: Introduction

A brain abscess is a focal, intracerebral infection characterized by a localized collection of pus within the brain parenchyma, resulting from microbial invasion and the body’s inflammatory response. It progresses through stages: early and late cerebritis, followed by early and late capsule formation, reflecting tissue destruction, immune response, and eventual encapsulation.Etiology and PathogenesisCausative organisms vary with source and host factors, often involving polymicrobial...
21
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...
15
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...
199
Cerebral Edema l: Introduction01:19

Cerebral Edema l: Introduction

Cerebral edema is a pathological increase in brain water content that disrupts intracranial pressure regulation and impairs neurological function. Because the cranial vault is rigid, even modest increases in tissue volume can compromise cerebral perfusion, distort neural structures, and initiate secondary injury. Cerebral edema develops through four principal mechanisms: vasogenic, cytotoxic, interstitial, and ionic.Vasogenic EdemaVasogenic edema arises from disruption of the blood–brain...
30