What are key findings from history in a child with a left-sided cerebellar tumor?
- Headaches for one year
- Increasing frequency of headaches
- Vomiting/falling at soccer
- Posterior location of headaches
What are key findings on physical exam of a child with a left-sided cerebellar tumor?
Ataxia, papilledema, left nystagmus, past-pointing (a pt. attempting to reach point of a finger will overshoot it)
What is on the differential diagnosis for left-sided cerebellar tumor?
Migraine, tension headache, stress reaction, brain tumor, sinusitis, toxin, basilar artery migraine
What is seen on the Brain MRI with a left-sided cerebellar tumor?
Enhancing mass within the left central portion of the cerebellum and evidence of obstructive hydrocephalus.
What is the epidemiology of brain tumors in children?
- Most common solid tumor in children
- Male incidence slightly higher than female
- Second most common form of childhood cancer (behind leukemia)
- Incidence is increasing for unknown reasons
What are risk factors for brain tumors in children?
- Exposure to ionizing radiation
- Certain genetic syndromes, such as:
- -Tuberous sclerosis
- -Li-Fraumeni syndrome
What are the typical symptoms of infratentorial lesions?
Usually present with cerebellar signs and sign of increased ICP.
What are the typical symptoms of cerebellar hemispheric lesions?
May see changes in muscle tone and deep tendon reflexes; more often find hypotonia and hyporeflexia.
What are typical symptoms of supratentorial lesions?
Focal motor and sensory abnormalities on side opposite the lesion.
What are typical symptoms of brain stem lesions?
Often associated with cranial nerve and gaze palsies.
What are the four histologic types of brain tumors?
- Primitive neuroectodermal tumor or medulloblastoma
- Brainstem glioma
What is a primitive neuroectodermal tumor or Medulloblastoma?
Most common of all pediatric brain tumors. Malignant tumor that can spread throughout nervous system. Capable of metastasizing to extracranial sites. Treatment and prognosis are dependent on size and dissemination of tumor. Treatment generally includes surgical resection, radiation and chemotherapy.
What is an astrocytoma?
Astrocytoma of the cerebellum has best prognosis of all infratentorial in children. Often with cystic component. Treatment is surgical resection, with five-year survival approx. 90 percent when completely resected. Radiation reserved for those with high-grade tumors, partial resections, or those in whom postoperative tumor progression is seen.
What is a brainstem glioma?
May be quite aggressive, resulting in diffuse infiltration of the pons, or low-grade, resulting in a focal tumor in the midbrain or medulla. Prognosis range from grave to good. Surgical resection alone required for low-grade gliomas.
What is an ependymoma?
Arises from within fourth ventricle (ependymal lining). Cause symptoms related to hydrocephalus. Treatment usually surgical resection plus radiation. Five-year survival approximately 50 percent.
What are the complications of brain tumor treatment?
- Deaths from brain tumors are highest among all childhood cancer deaths
- Long-term sequelae of childhood brain tumors are most often due to effects of chemotherapy and/or radiation therapy, including:
- Neurocognitive defects
- Attention deficit disorder
- Learning disabilities
- Endocrine abnormalities
What are tension headaches?
- Episodic, worsening throughout the day
- Mild to moderate intensity
- May feel like band around head or involve occipital area with tenderness of posterior muscles of the neck
- Occur in setting of emotional stress, fatigue, lack of sleep, and other stressors
What are migraine headaches?
- Most common cause of recurrent headache in children
- More severe than tension, and often throbbing
- May be accompanied by photophobia and/or phonophobia, abdominal pain, nausea, vomiting
- Precipitating factors include stress, bright lights, odors and foods
- Often relieved by sleep
What are the four migraine types?
- Basilar artery
- Migraine variants
What are Classic Migraines?
Accompanied by our (visual sx, speech changes, or other sensory abnormalities)
What are common migraines?
Most frequent migraine type in children. No aura, frequently unilateral (frontal or temporal)
What are basilar artery migraines?
Uncommon migraine variant associated with bilateral visual changes, paresthesias, and altered mental status.
What are migraine variants?
(seen exclusively in pediatric age group): Cyclical vomiting, abdominal migraines, benign paroxysmal vertigo.
What are concerning headache symptoms in a child?
- HA occur after a period of recumbency (eg early morning or after a nap) or awaken patient from sleep
- HA accompanied by - and relieved after - forceful vomiting
- Pain aggravated by bearing down (valsalva maneuvers)
- Sudden onset
- HA accompanied by photophobia and fever
- HA accompanied by elevation in bp, bradycardia, and irregular respirations (Cushing’s triad)
- Progressive increase in frequency and/or severity
- Responsible for coordination, precision, and controlling balance
- Mass lesions in the cerebellum often lead to obstructive hydrocephalus due to its close proximity to the fourth ventricle
- Knowledge of the function of the different parts of the cerebellum can help localize lesions
What do lesions in the vermis (midline) of the cerebellum cause?
Dysarthria, truncal ataxia, and gait abnormalities.
What do cerebellar hemispheric lesions cause?
Ipsilateral limb abnormalities, nystagmus, tremor/dysmetria. Patients fall toward side of the lesion and have worse nystagmus when looking toward side of lesion.
What do lesions of the deep cerebellar nuclei cause?
Resting tremor, myoclonus (muscle twitch)and opsoclonus (eye twitch)(such as seen in children with a neuroblastoma)
Term used to describe lack of coordination of muscle movements. This sign may result from dysfunction in various parts of nervous system, including cerebellum, the inner ear and the dorsal columns. May be congenital or acquired. A child with acute onset of ataxia requires immediate evaluation.
What are some etiologies of ataxia?
Post-infectious cerebellitis (acute cerebellar ataxia), Infectious cerebellitis, Medication or toxin, Intracranial mass, Opsoclonus-myoclonus syndrome, migraine headache, hydrocephalus, metabolic disease, neurodegenerative disease, psychiatric illness.
Post-infectious cerebellitis (acute cerebellar ataxia):
- Believed to be an autoimmune response to viral illness (such as varicella or coxcackie virus)
- Causes cerebellar demyelination
- Majority recover completely within a few months
Infectious cerebellitis (causing ataxia):
- May be bacterial or viral in origin (mumps, enteroviruses, EBV, Strep pneumo, neisseria meningitides, hemophilus influenzae B)
- Fever and mental status changes often observed
Medication or toxin (causing ataxia):
- Exposure to alcohol, antihistamines, or anti-convulsants
- May be accompanied by nystagmus and dysmetria
Intracranial mass (causing ataxia):
- Most often associated with tumor in the cerebellum or frontal lobe
- Associated findings depend on area of involvement
Opsoclonus-myoclonus syndrome (causing ataxia):
- Paraneoplastic syndrome that occurs most often with neuroblastoma
- Ataxia is accompanied by intermittent jerking movements (myoclonus) and erratic, jerky, conjugate movements of the eyes (opsoclonus)
Migraine headache (causing ataxia):
- Basilar artery migraines or hemiplegic migraines may cause ataxia
- Accompanying symptoms may include intermittent loss of vision, change in speech, headache, vomiting
Hydrocephalus (causing ataxia):
- Insidious onset, chronic - increasing loss of coordination over weeks to months
- Usually associated with headache and vomiting
Metabolic disease (causing ataxia):
- Associated with several metabolic disease, including maple syrup urine disease and pyruvate decarboxylase deficiency
- May be intermittent or chronic with intermittent exacerbations
Neurodegenerative disease (causing ataxia):
- Ataxia telangiectasia and Friedrich’s ataxia are most well known
- Additional symptoms include loss of developmental milestones and other neurological symptoms
Psychiatric illness (causing ataxia):
Conversion reaction can manifest as an hysterical involuntary gait disturbance.
- Cranial nerves and visual fields: test visual acuity, EOMI, pupillary reflex, facial sensation, and facial muscle movement and symmetry, position of uvula and tongue, symmetry and strength of sternocleidomastoid and trapezius muscles.
- Fundoscopic exam: Look for blurring of edge of optic disc and narrowing of vessels, evidence of optic disc swelling (papilledema). This finding is indicative of increased intracranial pressure.
- Sensation, motor strength, and deep tendon reflexes: test light touch, pinprick, and proprioception, symmetry of muscle strength and reflexes.
- Cerebellar function: Test finger to nose, heel to shin, and rapid alternating movements. Romberg sign: Patient unable to maintain steady upright posture while standing with arms extended anteriorly, palms upward and eyes closed or open.
- Plantar reflex: Check Babinski’s reflex (negative - downgoing)
Frequent upward rubbing of nose (salute) to alleviate itching leads to transverse lines across lower third of nose.
Infraorbital transverse creases associated with chronic conjunctival inflammation.
What is on the differential diagnosis for brain tumor?
Migraine, Tension headache, stress reaction, sinusitis, basilar migraine, toxin/medication side effect.
May occur any time of day, involve any part of the head, and is triggered by stress. A family history of migraines is present in 50 percent of those affected. The pattern is usually not progressive.
Often bilateral and involving forehead, temporal areas or back of head. Tenderness of the posterior muscles of the neck may be present. Generally worsen throughout the day. Stress can be a trigger.
May present with headache (tension or migraine) and/or difficulty concentrating. Other somatic complaints are also seen, including sleep difficulties, deteriorating school performance, and lack of interest in friends.
Likely to have focal neurologic findings. Signs of increased ICP, such as papilledema, are common with brain tumors, but not always present. An increase in severity and frequency of headaches is concerning. Worsening headache occurring in the morning associated with emesis and ataxia point strongly to a progressive problem, such as brain tumor.
One of the most common causes of headache. Poorly controlled allergic rhinitis may trigger exacerbations of sinusitis. Would likely also have fever, purulent nasal discharge, and sinus tenderness.
Vomiting, headache, and ataxia may all be seen in basilar migraine, but papilledema is a specific sign of increased intracranial pressure and makes migraine much less likely.
Toxin/medication side effect:
Ataxia is a side effect of many medications. Lateralizing signs on exam make a toxin less likely.
Provides excellent detail of the posterior fossa. In children, frequently requires sedation.
Will not visualize the posterior fossa well, but is often easier and faster to obtain than MI in some centers. May be valuable when intracranial hemorrhage needs to be ruled out.
When is Lumbar puncture contraindicated?
In patients with signs of increased ICP (may lead to brain herniation).
Management of headache:
Ask patient to keep a daily headache diary; Asking patient or parent to keep a written log of HA - including character and duration, location, associated symptoms, activity at the time of the headache, potential triggers, and actions that relieved the headache - can be a valuable clinical tool. The diary often includes more detail than can be recalled from memory alone.
Management and treatment of brain tumor:
Multidisciplinary approach is key. Requires immediate involvement of:
- Neurosurgeon: Surgery will establish histologic diagnosis and reduce tumor burden.
- Oncologist: Radiation therapy, chemotherapy, and other adjuvants (eg, bone marrow transplantation) may be indicated.
- Social worker: provides support and additional types of information
- PCP: Provides ongoing guidance and care throughout illness and in follow up