Syringobulbia

Original Editor - Shreya Pavaskar

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Description/Definition[edit | edit source]

Syringobulbia is a rare neurological disorder characterized by a fluid-filled cavity called syrinx within the spinal cord that extends to involve the caudal portion of the brainstem (especially the medulla). The sensory and motor neurons can be affected due to this interruption. This disorder can be associated with Syringomyelia (syrinx is limited to spinal cord) and Arnold-Chiari Malformation 1. “Communicating” syringomyelia/syringobulbia refers to cavities with direct communication with the fourth ventricle.

Medulla

Clinically Relevant Anatomy[edit | edit source]

The brainstem is an area that lies deep within the cerebral hemispheres and cervical spinal cord. It is divided into midbrain, pons and medulla oblongata. It controls the involuntary actions of the body like breathing. It has various control centers for actions such as swallowing and vasomotor control. It also has nuclei for cranial nerves, sympathetic and parasympathetic autonomic functions.[1]

Pathological Process[edit | edit source]

Syringobulbia is usually a late extension of syringomyelia into the medulla, although isolated cases of syringobulbia have been documented[2]. The exact etiology is unknown. However some factors may be considered - small posterior fossa and birth trauma leading to hematomyelia or arachnoid scarring[3]. Jonesco-Sisesti described cavitation in 3 spaces - motor and sensory nuclei in the floor of the fourth ventricle and extending anterolaterally from there (most common), midline from the floor of the fourth ventricle along the medial raphe for a shorter or longer distance and also medial and ventral, interrupting fibers of the hypoglossal nerve.[4]

Clinical Presentation[edit | edit source]

Damage is caused due to cavity (syrinx) or a slit like structure called clefts. The presentation isn't same in all patients and may vary depending upon the location of the syrinx in medulla and the severity.

The most common signs and symptoms are [5]

  • Diplopia
  • Episodic vertigo
  • Trigeminal distribution pain
  • Dysarthria
  • Hoarseness in voice
  • Unilateral tongue atrophy
  • Analgesia and loss of temperature sensation on one side of the face
  • Palatal and vocal cord paralysis
  • Absent gag reflex
  • Nystagmus
  • Dysphonia
  • Horner's Syndrome

Patients with Syringomyelia may present suddenly with hearing loss or ringing in the ears (tinnitus) , ataxia, trigeminal nerve pain, nausea and vomiting.[3]

The symptoms are almost always unilateral and the clefts are frequently bilateral.[6][7]

Occasionally, Medulla can show great adaptation to slowly growing cysts and clefts thus a patient with clefts in medulla may or may not show symptoms owing to adaptation and neural plasticity.[3]

The visceral and autonomic manifestations include -

  • Tachycardia
  • Cardiac arrhythmias
  • Orthostatic hypotension
  • Disturbances in respiratory rhythm during wakefulness and sleep

[8]


Diagnostic Procedures[edit | edit source]

A neurological exam should rule out most of the symptoms and other neurological conditions. The diagnosis is made by the means of neuroimaging mainly MRI (Magnetic Resonance Imaging).

Medical Management[edit | edit source]

There is no specific procedure for the treatment of Syringobulbia however the causes can be met if feasible, for example, tumor removal can be performed.

Physiotherapy Management[edit | edit source]

The physiotherapy management is similar to Syringomyelia. Each treatment protocol is different for different patient depending upon the symptoms.

Check out Syringomyelia page on Physiopedia for management - Syringomyelia

References[edit | edit source]

  1. Robert Joynt, Brainstem, Encyclopaedia Britannica
  2. Sahoo S, Pearl PL. Syringobulbia. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:590-91.
  3. 3.0 3.1 3.2 Nogués M, López L, Meli F. Neuro-ophthalmologic complications of syringobulbia. Current neurology and neuroscience reports. 2010 Nov 1;10(6):459-66.
  4. Murray TJ. SYRINGOBULBIA—A CONTRIBUTION TO THE PATHOPHYSIOLOGY OF THE BRAINSTEM. 1986. By N. Jonesco-Sisesti. Translated into English, edited and annotated by Dr. Robert T. Ross. Published by Praeger, New York and London. 315 pages. $65 Cdn. approx. Canadian Journal of Neurological Sciences. 1987 Nov;14(4):659-.
  5. Roser F. Syringomyelia and Syringobulbia. InSamii's Essentials in Neurosurgery 2014 (pp. 569-576). Springer, Berlin, Heidelberg.
  6. Williams B: Simultaneous cerebral and spinal fluid pressure recordings. 2. Cerebrospinal dissociation with lesions at the foramen magnum. Acta Neurochir (Wien) 1981, 59:123–142.
  7. Nogués M. Syringomyelia and syringobulbia. In Handbook of Clinical Neurology, vol 50. Edited by Vinken PJ, Bruyn GW. Amsterdam: Elsevier; 1987:443–464
  8. JMedical Condition Information. Syringobulbia (Medical Condition). Available from: http://www.youtube.com/watch?v=xxUYju9Q-QY [last accessed 28/12/2020]