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Related Concept Videos

Cranial and Spinal Meninges01:19

Cranial and Spinal Meninges

The cranial and spinal meninges are complex protective structures surrounding the central nervous system (CNS), consisting of the brain and spinal cord. These meninges consist of the dura mater, the arachnoid mater, and the pia mater. They protect the CNS, provide structural support, and aid in circulating cerebrospinal fluid (CSF).
Cranial Meninges
These meningeal layers cover the cranium. The dura mater is the outermost layer of cranial meninges. It is a thick and durable membrane of dense...
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...
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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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...
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...
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Cryptococcal Meningitis

Cryptococcal meningitis is a life-threatening opportunistic infection predominantly associated with HIV/AIDS, accounting for over 100,000 deaths annually worldwide. However, it also affects individuals with other forms of immunosuppression, including those undergoing immunosuppressive therapy, organ transplant recipients, patients with innate immunodeficiencies, and individuals with hematological disorders. The infection is caused mainly by Cryptococcus neoformans and Cryptococcus gattii,...

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Proteus syndrome complicated by multiple spinal meningiomas.

A Asahina1, H Fujita, T Omori

  • 1Department of Dermatology, Sagamihara National Hospital, Sagamihara, Kanagawa, Japan. asahina-tky@umin.ac.jp

Clinical and Experimental Dermatology
|August 7, 2008
PubMed
Summary

Proteus syndrome, a rare disorder, can present with unusual complications like spinal meningiomas, mimicking other conditions. Early recognition of its diverse manifestations is crucial for accurate diagnosis and management.

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Area of Science:

  • Genetics and rare diseases
  • Clinical case study
  • Medical diagnostics

Background:

  • Proteus syndrome is a rare congenital disorder characterized by mosaic overgrowth and malformations affecting multiple tissues.
  • Accurate diagnosis can be challenging due to its high variability and infrequent presentation of certain complications.
  • Spinal meningiomas are an exceptionally rare manifestation, potentially leading to misdiagnosis.

Observation:

  • A 65-year-old Japanese man with Proteus syndrome presented with spinal meningiomas and neural symptoms.
  • He also exhibited right leg hypertrophy, sole cerebriform connective-tissue naevus, and macrodactyly.
  • Mild cystic and emphysematous lung changes were noted on chest CT, possibly linked to the syndrome.

Findings:

  • The patient's presentation, particularly the spinal meningiomas, complicated the diagnosis, initially suggesting neurofibromatosis.
  • Despite unusual features, the patient had survived to an advanced age due to otherwise mild clinical manifestations.
  • This case highlights the diagnostic challenges posed by atypical Proteus syndrome presentations.

Implications:

  • Increased awareness of Proteus syndrome's diverse clinical spectrum, including rare complications like spinal meningiomas, is essential for clinicians.
  • Timely and accurate diagnosis of Proteus syndrome facilitates appropriate management and patient care.
  • Further research into the genetic underpinnings and varied manifestations of Proteus syndrome is warranted.