Case 20

Question 1

What are key findings from history in a child with a left-sided cerebellar tumor?

Answer 1

• Headaches for one year
• Increasing frequency of headaches
• Vomiting/falling at soccer
• Posterior location of headaches

Question 2

What are key findings on physical exam of a child with a left-sided cerebellar tumor?

Answer 2

Ataxia, papilledema, left nystagmus, past-pointing (a pt. attempting to reach point of a finger will overshoot it)

Question 3

What is on the differential diagnosis for left-sided cerebellar tumor?

Answer 3

Migraine, tension headache, stress reaction, brain tumor, sinusitis, toxin, basilar artery migraine

Question 4

What is seen on the Brain MRI with a left-sided cerebellar tumor?

Answer 4

Enhancing mass within the left central portion of the cerebellum and evidence of obstructive hydrocephalus.

Question 5

What is the epidemiology of brain tumors in children?

Answer 5

• 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

Question 6

What are risk factors for brain tumors in children?

Answer 6

• Exposure to ionizing radiation
• Certain genetic syndromes, such as:
• -Tuberous sclerosis
• -Neurofibromatosis
• -Li-Fraumeni syndrome

Question 7

What are the typical symptoms of infratentorial lesions?

Answer 7

Usually present with cerebellar signs and sign of increased ICP.

Question 8

What are the typical symptoms of cerebellar hemispheric lesions?

Answer 8

May see changes in muscle tone and deep tendon reflexes; more often find hypotonia and hyporeflexia.

Question 9

What are typical symptoms of supratentorial lesions?

Answer 9

Focal motor and sensory abnormalities on side opposite the lesion.

Question 10

What are typical symptoms of brain stem lesions?

Answer 10

Often associated with cranial nerve and gaze palsies.

Question 11

What are the four histologic types of brain tumors?

Answer 11

1. Primitive neuroectodermal tumor or medulloblastoma
2. Astrocytoma
3. Brainstem glioma
4. Ependymoma

Question 12

What is a primitive neuroectodermal tumor or Medulloblastoma?

Answer 12

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.

Question 13

What is an astrocytoma?

Answer 13

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.

Question 14

What is a brainstem glioma?

Answer 14

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.

Question 15

What is an ependymoma?

Answer 15

Arises from within fourth ventricle (ependymal lining). Cause symptoms related to hydrocephalus. Treatment usually surgical resection plus radiation. Five-year survival approximately 50 percent.

Question 16

What are the complications of brain tumor treatment?

Answer 16

• 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
• Stroke

Question 17

What are tension headaches?

Answer 17

• 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

Question 18

What are migraine headaches?

Answer 18

• 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

Question 19

What are the four migraine types?

Answer 19

• Classic
• Common
• Basilar artery
• Migraine variants

Question 20

What are Classic Migraines?

Answer 20

Accompanied by our (visual sx, speech changes, or other sensory abnormalities)

Question 21

What are common migraines?

Answer 21

Most frequent migraine type in children. No aura, frequently unilateral (frontal or temporal)

Question 22

What are basilar artery migraines?

Answer 22

Uncommon migraine variant associated with bilateral visual changes, paresthesias, and altered mental status.

Question 23

What are migraine variants?

Answer 23

(seen exclusively in pediatric age group): Cyclical vomiting, abdominal migraines, benign paroxysmal vertigo.

Question 24

What are concerning headache symptoms in a child?

Answer 24

• 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

Question 25

Cerebellum:

Answer 25

- 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

Question 26

What do lesions in the vermis (midline) of the cerebellum cause?

Answer 26

Dysarthria, truncal ataxia, and gait abnormalities.

Question 27

What do cerebellar hemispheric lesions cause?

Answer 27

Ipsilateral limb abnormalities, nystagmus, tremor/dysmetria. Patients fall toward side of the lesion and have worse nystagmus when looking toward side of lesion.

Question 28

What do lesions of the deep cerebellar nuclei cause?

Answer 28

Resting tremor, myoclonus (muscle twitch)and opsoclonus (eye twitch)(such as seen in children with a neuroblastoma)

Question 29

Ataxia:

Answer 29

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.

Question 30

What are some etiologies of ataxia?

Answer 30

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.

Question 31

Post-infectious cerebellitis (acute cerebellar ataxia):

Answer 31

- 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

Question 32

Infectious cerebellitis (causing ataxia):

Answer 32

- May be bacterial or viral in origin (mumps, enteroviruses, EBV, Strep pneumo, neisseria meningitides, hemophilus influenzae B) - Fever and mental status changes often observed

Question 33

Medication or toxin (causing ataxia):

Answer 33

- Exposure to alcohol, antihistamines, or anti-convulsants | - May be accompanied by nystagmus and dysmetria

Question 34

Intracranial mass (causing ataxia):

Answer 34

- Most often associated with tumor in the cerebellum or frontal lobe - Associated findings depend on area of involvement

Question 35

Opsoclonus-myoclonus syndrome (causing ataxia):

Answer 35

- 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)

Question 36

Migraine headache (causing ataxia):

Answer 36

- Basilar artery migraines or hemiplegic migraines may cause ataxia - Accompanying symptoms may include intermittent loss of vision, change in speech, headache, vomiting

Question 37

Hydrocephalus (causing ataxia):

Answer 37

- Insidious onset, chronic - increasing loss of coordination over weeks to months - Usually associated with headache and vomiting

Question 38

Metabolic disease (causing ataxia):

Answer 38

- Associated with several metabolic disease, including maple syrup urine disease and pyruvate decarboxylase deficiency - May be intermittent or chronic with intermittent exacerbations

Question 39

Neurodegenerative disease (causing ataxia):

Answer 39

- Ataxia telangiectasia and Friedrich's ataxia are most well known - Additional symptoms include loss of developmental milestones and other neurological symptoms

Question 40

Psychiatric illness (causing ataxia):

Answer 40

Conversion reaction can manifest as an hysterical involuntary gait disturbance.

Question 41

Neurologic Exam:

Answer 41

- 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)

Question 42

Allergic salute:

Answer 42

Frequent upward rubbing of nose (salute) to alleviate itching leads to transverse lines across lower third of nose.

Question 43

Dennie's lines:

Answer 43

Infraorbital transverse creases associated with chronic conjunctival inflammation.

Question 44

What is on the differential diagnosis for brain tumor?

Answer 44

Migraine, Tension headache, stress reaction, sinusitis, basilar migraine, toxin/medication side effect.

Question 45

Migraine:

Answer 45

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.

Question 46

Tension headache:

Answer 46

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.

Question 47

Stress reaction:

Answer 47

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.

Question 48

Brain tumor:

Answer 48

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.

Question 49

Sinusitis:

Answer 49

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.

Question 50

Basilar migraine:

Answer 50

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.

Question 51

Toxin/medication side effect:

Answer 51

Ataxia is a side effect of many medications. Lateralizing signs on exam make a toxin less likely.

Question 52

MRI:

Answer 52

Provides excellent detail of the posterior fossa. In children, frequently requires sedation.

Question 53

CT:

Answer 53

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.

Question 54

When is Lumbar puncture contraindicated?

Answer 54

In patients with signs of increased ICP (may lead to brain herniation).

Question 55

Management of headache:

Answer 55

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.

Question 56

Management and treatment of brain tumor:

Answer 56

Multidisciplinary approach is key. Requires immediate involvement of: - Neurologist - 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