Diffuse intrinsic pontine glioma: DIPG


Case presentation

A 4-year-old female presents with the parents noting that the child has been “not walking right” and “wobbly” for the past 3 weeks. She sustained a head injury 1 month prior to her current presentation but there was no reported loss of consciousness at that time and the fall was off a chair, onto a hardwood floor, less than 2 feet. At the time, she did not appear injured and had been at her baseline until the onset of current symptoms. There has been no reported fever, recent illness, emesis, neck pain, back pain, or incontinence. The family has noted that her “eyes look funny” and she appears to “have trouble focusing her vision.” She also has been drooling (new for this child) and seems to have difficulty speaking, which is also new for the child as she has been developmentally appropriate.

Physical examination reveals age-appropriate vital signs and the child is in no overall distress. She is normocephalic and there are no signs of trauma. Pupils are round and equally reactive to light and there is no photophobia. The child has bilateral ptosis and she appears to have difficulty focusing her vision on objects. She has equal tone and strength throughout, but when she ambulates, she is ataxic and has dysmetria. She is drooling and is difficult to understand when she speaks.

Imaging considerations

Imaging is often employed in patients with general neurologic complaints, such as severe headache, and focal neurologic findings (such as ataxia or abnormal neurologic examination), or if there are symptoms that raise suspicion for an intracranial process, such as emesis, vision changes, or concerning historical findings (such as awakening in the middle of the night with headache, first morning emesis, or persistent/worsening headache). Imaging options include computed tomography (CT) and magnetic resonance imaging (MRI); which modality is employed depends largely on history, physical examination, and institutional resources.

While the use of neuroimaging has become commonplace for the evaluation of a variety of symptoms in pediatric patient populations, including concussion, , first-time seizures, , headache, , and dizziness, , neuroimaging may not be indicated in all patients. Several studies looking at acute and subacute ataxia in pediatric patients found conditions that required intervention in approximately 13% of patients imaged. , Of note, the clinical features most predictive of imaging abnormalities were the age of the child (older children had more significant intracranial pathology), the duration of symptoms (more than 3 days was significant), and the presence of additional neurological signs on examination, such as cranial nerve deficits or hemiparesis. , Cranial nerve deficits are associated with a variety of intracranial pathologies, including demyelinating disease and tumors; more than 80% of children with space-occupying tumors will have signs of increased intracranial pressure, in addition to ataxia. , Therefore it is a constellation of symptoms and examination findings that leads the clinician to suspect an intracranial process, rather than individual symptoms alone.

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