Diagnostic dilemma: A visual representation of the challenge in differentiating between GBS and SSEH.

Guillain-Barré Syndrome or Something Else? A Case Study You Need to Know

"When rare conditions mimic common illnesses, early detection is key. Learn how one case highlights the diagnostic challenges and importance of swift action in neurological emergencies."


Imagine a child suddenly unable to walk, pain shooting through their legs, reflexes disappearing – the mind immediately jumps to Guillain-Barré Syndrome (GBS). GBS, an autoimmune disorder affecting the peripheral nerves, can indeed present with these frightening symptoms. But what if it's not GBS? What if it's something far rarer, yet equally devastating, mimicking the well-known signs?

This is the unsettling reality highlighted in a recent case report published in 'Brain & Development.' The study details the story of a young girl whose initial symptoms strongly suggested GBS, leading doctors down a specific treatment path. However, as time ticked by, and her condition worsened, it became clear that a different, much rarer culprit was at play: a spontaneous spinal epidural hematoma (SSEH).

SSEH, a neurological emergency involving bleeding into the space surrounding the spinal cord, can compress the delicate neural tissue, leading to paralysis, sensory loss, and even respiratory failure. The challenge lies in its ability to masquerade as more common conditions like GBS, potentially delaying crucial treatment and impacting patient outcomes.

The Case of the Mimicking Symptoms: How a Spinal Hematoma Fooled Initial Diagnosis

Diagnostic dilemma: A visual representation of the challenge in differentiating between GBS and SSEH.

A previously healthy six-year-old girl arrived at the emergency department with a troubling two-day history. She complained of increasing pain in her lower extremities and was unable to walk. This followed a neck injury she sustained a month earlier, although it didn't immediately seem serious. A week before her hospital visit, she also had a severe cough and fever.

Upon examination, the girl was alert but in significant pain. She was completely unable to move her legs (paraparetic), and her deep tendon reflexes were absent in her lower extremities. Babinski reflexes, which can indicate central nervous system issues, were present on both sides. She also showed a reduced response to touch and pinprick below the T10 dermatome level and had difficulty urinating. The strength, sensation, and movement in her upper extremities remained normal.

  • Initial Suspicion of GBS: Based on the symptoms and initial findings, doctors suspected GBS. A lumbar puncture was performed, and while initial MRI scans of the lower spine came back normal, the cerebrospinal fluid (CSF) showed elevated protein levels – a hallmark of GBS.
  • Treatment Begins: Intravenous immunoglobulin (IVIG) therapy, the standard treatment for GBS, was initiated.
  • Condition Worsens: Despite treatment, the girl's condition didn't improve. The next day, she developed pain and weakness in her right upper extremity. Three days after admission, her respiratory function rapidly declined.
It was then that an emergency spinal MRI revealed the true cause: a mass extending from the C7 to T3 vertebrae, compressing her spinal cord. The girl underwent an emergency laminectomy to evacuate the hematoma. Doctors discovered a dark brown blood clot, indicating chronic venous bleeding. She was diagnosed with SSEH. Although she received further treatment, including methylprednisolone, she was left with severe neurologic sequelae of paralysis in both legs.

Lessons Learned: Why Awareness is Key

This case underscores the critical importance of considering rare conditions in the differential diagnosis, even when initial symptoms point towards a more common illness. SSEH, though rare, carries severe consequences, including permanent neurological deficits and even death. The case highlights that while GBS remains a significant concern, clinicians must maintain a high index of suspicion for other potentially reversible conditions that can mimic its presentation, such as SSEH. If a patient diagnosed with GBS presents with atypical signs, such as positive Babinski reflexes, or if their condition deteriorates rapidly despite appropriate treatment, further investigation, including spinal imaging, is warranted. Early recognition and prompt surgical intervention are critical to improving outcomes in patients with SSEH.

About this Article -

This article was crafted using a human-AI hybrid and collaborative approach. AI assisted our team with initial drafting, research insights, identifying key questions, and image generation. Our human editors guided topic selection, defined the angle, structured the content, ensured factual accuracy and relevance, refined the tone, and conducted thorough editing to deliver helpful, high-quality information.See our About page for more information.

This article is based on research published under:

DOI-LINK: 10.1016/j.braindev.2018.11.004, Alternate LINK

Title: Spontaneous Spinal Epidural Hematoma Mimicking Guillain-Barre Syndrome

Subject: Neurology (clinical)

Journal: Brain and Development

Publisher: Elsevier BV

Authors: Aya Kondo, Hiroshi Yamaguchi, Yusuke Ishida, Daisaku Toyoshima, Mai Azumi, Nobuyuki Akutsu, Junji Koyama, Hiroshi Kurosawa, Atushi Kawamura, Azusa Maruyama

Published: 2019-04-01

Everything You Need To Know

1

What is Guillain-Barré Syndrome (GBS), and why is it important to recognize?

Guillain-Barré Syndrome (GBS) is an autoimmune disorder where the body's immune system attacks the peripheral nerves. This can lead to muscle weakness, paralysis, and sometimes respiratory failure. It's significant because it can be life-threatening and requires prompt diagnosis and treatment to minimize nerve damage and support bodily functions until recovery occurs. The symptoms can sometimes be similar to other conditions making it a challenge to diagnose.

2

What is a spontaneous spinal epidural hematoma (SSEH), and why is it considered a neurological emergency?

A spontaneous spinal epidural hematoma (SSEH) is a rare condition involving bleeding into the space surrounding the spinal cord. This bleeding can compress the spinal cord, leading to neurological deficits like paralysis, sensory loss, and respiratory failure. It is considered a neurological emergency, it requires rapid diagnosis and intervention, usually surgical, to relieve the pressure on the spinal cord and prevent permanent damage. Early detection is key in cases where the symptoms mirror other conditions.

3

How can you tell the difference between SSEH and Guillain-Barré Syndrome (GBS) based on symptoms?

The symptoms of SSEH can resemble those of Guillain-Barré Syndrome (GBS), such as leg weakness, pain, and loss of reflexes. A key difference lies in additional signs. Rapid deterioration despite GBS treatment or atypical signs should raise suspicion for SSEH. Early clues can include positive Babinski reflexes, rapid decline of respiratory function and the nature of the pain experienced. Prompt investigation with spinal imaging, like MRI, is crucial to differentiate between the two.

4

What is IVIG, and why is it used in the context of Guillain-Barré Syndrome (GBS)?

IVIG, or Intravenous Immunoglobulin therapy, is a standard treatment for Guillain-Barré Syndrome (GBS). It involves administering antibodies intravenously to help modulate the immune system and reduce the attack on the peripheral nerves. In the context of conditions mimicking GBS, like SSEH, IVIG would not be effective, and the patient's condition might worsen, highlighting the importance of accurate diagnosis to ensure appropriate treatment.

5

Why is early diagnosis so crucial when dealing with conditions that mimic Guillain-Barré Syndrome (GBS)?

Early diagnosis is extremely important because conditions like spontaneous spinal epidural hematoma (SSEH) can cause permanent neurological damage or even death if not treated promptly. In the case of SSEH, surgical intervention to evacuate the hematoma is often necessary to relieve pressure on the spinal cord. Delayed diagnosis, especially when SSEH mimics Guillain-Barré Syndrome (GBS), can lead to irreversible damage, underscoring the need for clinicians to consider rare conditions in their differential diagnosis.

Newsletter Subscribe

Subscribe to get the latest articles and insights directly in your inbox.