Syringobulbia. Report of a case.

J Med Assoc Thai

Published: November 1972

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Gowers Intrasyringeal Hemorrhage with Syringomyelia and Syringocephaly.

Neurol India

September 2024

Departments of Radiodiagnosis, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India.

A 30-year-old man patient presented with the progressive weakness and pain in the back, arms and legs, sensory loss, facial weakness, diplopia, difficulty of speech and swallowing since 4 months. MRI revealed syringomyelia and syringocephaly involving medulla (syringobulbia), pons (syringopontia) and midbrain (syringomesencephaly) with intrasyringeal hemorrhage.

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Background: Unlike syringomyelia, syringobulbia is not commonly observed in pediatric patients with Chiari malformation type I (CMI). Previous series have reported the incidence of syringobulbia as between 3% and 4% in these patients. Presentation is typically chronic, with the slow onset of neurological symptoms and cranial nerve (CN) palsies resulting from lower brainstem involvement.

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Article Synopsis
  • - Chiari malformation type I (CM-I) involves cerebellar tonsils herniating into the spinal canal, which can lead to serious complications like cerebrospinal fluid flow blockage and brainstem pressure, particularly worsened by stressors like COVID-19.
  • - A 44-year-old woman with CM-I experienced severe symptoms after violent coughing due to COVID-19, including intense headaches, bulbar symptoms, and severe sleep-disordered breathing, resulting in respiratory failure that required mechanical ventilation.
  • - Surgical decompression relieved her symptoms over six months, highlighting the importance of prompt treatment for CM-I, especially as COVID-19 can trigger and intensify existing complications, risking severe outcomes like coma or death.
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Background: Intractable hiccups (IH) due to syringomyelia or syringomyelia/syringobulbia associated with Chiari type I malformations (CMI) are extremely rare. Here, we present two patients who presented with IH; one had a CMI with syringomyelia/syringobulbia, and the other, with CMI and syringomyelia.

Case Description: The first patient was an 18-year-old female who presented with IH attributed to a holocord syrinx and syringobulbia involving the right dorsolateral medulla.

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Background: Chiari I malformation (Chiari I) is defined by the downward displacement of one or both cerebellar tonsils below the foramen magnum of the skull with crowding altering cerebrospinal fluid flow. It can be associated with the development of a fluid-filled cavity within the spinal cord, syringomyelia. Neurological deficits or symptoms can occur at the level of anatomic involvement of syringomyelia.

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