Table 2 Salient clinical and imaging features of brainstem lesions in the paediatric age group
From: Paediatric brainstem: A comprehensive review of pathologies on MR imaging
Aetiology | Clinical features | Imaging features |
|---|---|---|
Vascular | ||
Infarction | Acute neurological deficit Bulbar symptoms to comatose state Associated conditions: cardiac disease, haemolytic anaemia or chemotherapy related | T2/FLAIR hyperintensity + diffusion restriction |
Hypoxic ischaemic encephalopathy | Birth asphyxia Obstructed labour Poor Apgar score | Typical sites of involvement: ventrolateral thalamus, posterior limb of the internal capsule and peri-Rolandic cortex. Dorsal brainstem involvement suggests profound hypoxia |
Vascular malformations | Asymptomatic Acute neurological deficit in acute bleed Progressive neurological deficits | Cavernous angioma: “popcorn” appearance Developmental venous anomaly: “caput medusae” appearance |
Vasculitis (Behçet’s disease) | Peak in 2nd decade Multisystem disorder involving the oral cavity, genitalia and eyes Skin, joints and other major systems may also be involved | T2 hyperintense lesions with variable post-contrast enhancement Sites: brainstem > basal ganglia > thalamus |
Demyelination | ||
ADEM | Recent vaccination or viral illness Monophasic disease | Asymmetric T2-hyperintense subcortical white matter lesion + basal ganglia and thalami involvement Brainstem involvement: uncommon. Isolated brainstem involvement: extremely rare |
Multiple sclerosis | More common in 2nd decade with female preponderance Progressive disease with chronic relapsing and remitting course | Posterior fossa and brainstem involvement frequent in paediatric age group |
Neuromyelitis optica | Optic neuritis Clinical features of long segment myelitis | T2 hyperintense lesions characteristically in periventricular location around the third, fourth ventricles and midbrain and cerebellum Increased signal intensity in the optic nerves, chiasma as well as the spinal cord |
Osmotic demyelination | Electrolyte disturbances Rapid correction of sodium Malnutrition Transplantation | T2 hyperintense signal in the central pons with characteristic sparing of the ventrolateral pons and cerebrospinal tracts Basal ganglia and thalami may be involved |
Metabolic, toxic and neurodegenerative diseases | ||
Leigh syndrome | Typical age group <2 years Hypotonia Neurological regression Elevated serum or CSF lactate levels | T2 hyperintense signal in basal ganglia, periaqueductal white matter, medulla and midbrain Diffusion restriction in acute states Lactate peak in affected regions |
Maple syrup urine disease | Neonatal onset Lethargy Seizures Characteristic urine odour | Diffuse brain swelling Diffusion restriction in posterior brainstem tracks and the central cerebellar white matter |
Wilson’s disease | Signs of hepatic involvement KF ring Extrapyramidal signs Raised serum ceruloplasmin | Basal ganglia and thalami typically involved in symmetrical pattern Midbrain and pons involvement can be seen Double panda sign |
Haemolytic uremic syndrome | History of diarrhoeal infection followed by acute renal failure, thrombocytopenia and haemolytic anaemia | Basal ganglia especially dorsolateral lentiform nuclei involvement + arterial infarctions + patterns of reversible encephalopathy |
Posterior reversible encephalopathy syndrome | Elevated blood pressure Children with renal failure or on chemotherapeutic agents | Typically involves parieto-occipital cortex or subcortical white matter. Atypical cases may involve the brainstem and basal ganglia |
Central tegmental tract lesions | Non-specific finding seen in various conditions such as cerebral palsy, metabolic disorders and drug toxicity | Symmetrical T2-hyperintense foci involving the midbrain, pontine or medullary tegmentum |
Brainstem encephalitis | ||
Viral encephalitis | Fever, altered sensorium Bulbar symptoms History of canine bite in suspected rabies encephalitis | Herpes encephalitis: bilateral/unilateral temporal lobe ± brainstem involvement Japanese encephalitis: bilateral thalamic with brainstem involvement Rabies: cord, brainstem, basal ganglia, hippocampal and hypothalamic involvement |
Tuberculosis | Hydrocephalus CSF analysis Immunocompromised patients | Basal meningitis, variable signal intensity of granuloma, ring enhancement |
Fungal | Immunocompromised patients Sinus disease | Haemorrhages, infarctions secondary to angioinvasive nature |
Cerebral malaria | Fever Altered sensorium Endemic regions | Multiple acute infarcts affecting various parts of the brain |
Tumours | ||
Brainstem glioma | Most common in 1st decade, peak between 3–7 years Signs of raised intracranial tension Long tract signs with cranial nerve palsies | Pontine glioma: non- enhancing expansive lesion, typically engulfs basilar artery, flat fourth ventricle sign Tectal glioma: non-enhancing lesions ± obstructive hydrocephalus Medullary glioma: focal, exophytic or diffuse lesion. Variable enhancement pattern |
Neurofibromatosis I | Family history Cutaneous stigmata such as café au lait spots, neurofibromas, etc. | Non-neoplastic brainstem enlargement T2 hyperintense lesions—hamartoma or glioma Aqueductal stenosis and hydrocephalus |