Introduction
Diseases of the paediatric brainstem |
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Vascular: |
Infarction |
Haemorrhage |
Hypoxia |
Vasculitis |
Vascular malformations |
Demyelination: |
ADEM |
Multiple sclerosis |
Neuromyelitis optica |
Osmotic demyelination |
Metabolic and neurodegenerative: |
Leigh’s disease |
Maple syrup urine disorder |
Glutaric aciduria |
Wilson’s disease |
Encephalitis: |
Viral |
Tubercular |
Fungal |
Parasitic |
Tumours: |
Pontine glioma |
Tectal glioma |
Medullary glioma |
Vascular Disorders of the Brainstem
Stroke
Hypoxic Ischaemic Injury
Vasculitis (Behçet’s Disease)
Vascular Malformations of the Brainstem
Demyelinating Pathologies
Acute Disseminated Encephalomyelitis
Multiple Sclerosis
Neuromyelitis Optica (NMO)
Osmotic Demyelination
Metabolic and Neurodegenerative Diseases
Leigh Syndrome
Maple Syrup Urine Disease (MSUD)
Wilson’s Disease
Haemolytic Uraemic Syndrome (HUS)
Posterior reversible encephalopathy syndrome
Central tegmental tract (CTT) lesions
Brainstem encephalitis
Viral Encephalitis
Tuberculosis and Fungal
Parasitic (Cerebral Malaria)
Tumours of the Brainstem
Brainstem Glioma
Secondary involvement of paediatric brainstem
Conclusion
Aetiology | Clinical features | Imaging features |
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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 |