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Brain Stem Glioma

 

 

 

Signs & Symptoms

Duration of symptoms is highly variable (weeks to years prior to the diagnosis) and depends on the type of tumor.

  • Diffuse gliomas have a shorter history of neurologic symptoms.  In one study the mean duration of symptoms prior to diagnosis was 2.6 months with 77% of patients having at least on cranial nerve abnormality.
  •  Focal tumors are associated with more prolonged symptoms.  On average symptoms are present for 10.6 months prior to diagnosis and cranial nerve involvement is only seen in 29%.

 

Most Common Signs of Brain Stem Glioma:

  • Palsy of the soft palate
  • Facial palsy
  • Pyramidal weakness.
  • Gait ataxia
  • Dysarthria
  • Difficulty swallowing are also frequently observed. 

 

Tectal Lesions

  • Often accompanied by nausea and vomiting. 
  • Diplopia (due to involvement of the medial longitudinal fasciculus).
  • Nystagmus, oculomotor paresis, and eyelid retraction are also symptoms associated with these tumors (involvement of cranial nerves III and IV). 

Signs of increased intracranial pressure

  • Less common in children than in adults. 
  • Papilledema = optic disc swelling secondary to raised intracranial pressure.
  • Headaches and hydrocephalus tend to occur when tumor involves the periaqueductal or fourth ventricle outflow areas.

Medullary involvement

  • Causes  bulbar signs, which include difficulties in swallowing and feeding.
  • Other signs include asymmetry of the palate, an absent gag reflex, atrophy of the tongue, and nasal regurgitation of food.

 

This 2 year old boy presented with a short history (one to two months only) of lower limb weakness, difficulty swallowing and signs of a respiratory tract infection. The sagittal T1 MR below shows a tumor (#1) expanding the medulla oblongata. The lesion is pushing the pons superiorly.

 

This is the same tumor (#1) below - it has high intensity signal on T2 axial scan.

Below the MR shows that this tumor (#1) has early enhancement on post Gadolinium T1 axial scan.

 

This tumor was a high grade diffuse brain stem glioma.

 

Cerebellar dysfunction

  • Linked to motor abnormalities.
  • Limb ataxia is a common symptom.
  • These motor difficulties may also reflect the involvement of the corticospinal tracts.

 

Infants affected by a pontine tumors

  • Often appear emaciated and have profound failure to thrive.
  • May have facial paresis.
  • Often lack the cough response during suctioning, display a depressed gag reflex, and exhibit respiratory irregularities. 

               

    Personality changes may also be observed in older children with pontine tumors.

Table :   Brain areas involved and associated symptoms

Brain area involved Associated Symptom(s)
Tectum

Headache

Nausea

Vomiting

Diplopia

Eyelid retraction

Nystagmus
Cervicomedullary area Loss of sensation in the face

Dysphagia

Ataxia

Oculomyoclonus
Medulla

Difficulties in swallowing and feeding

Asymmetry of the palate

Absent gag reflex

Atrophy of the tongue

Nasal regurgitation of food
Cerebellum Limb ataxia
Ventricular locations Hydrocephalus
Pons

All of the above, as well as:

Palatal palsy

Dysarthria

Drowsiness

Head tilt

Deterioration of hand writing and speech

 

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