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medU | Instructors

Case 20
Author: Mary Moran, M.D., MCP Hahnemann School of Medicine

Learning Objectives
1. List key features in the history of a child with headaches.
2. List the elements of a thorough neurologic exam and interpret
3. Discuss the differential diagnosis of headaches in children.
4. Discuss the differential diagnosis of ataxia in children.
5. Identify signs and symptoms of increased intracranial pressure.
6. List things to consider when conveying difficult news to a patient and family.
Summary of clinical scenario: Seven-year-old Nick is in the clinic because his
headaches, which he has had monthly for a year, are increasing in frequency. The
history reveals that his mother suffers from migraines and that Nick's mother and
father have recently separated. Ten days after the initial visit, Nick returns with
increasingly frequent and severe headaches and additional signs and symptoms
that include papilledema, horizontal nystagmus to the left, an ataxic gait, left leg
hyporeflexia and hypotonia, and a positive Romberg test. He is diagnosed with a
left-sided cerebellar tumor.
First network:
Family history of migraines
Headaches for one year
Stress at home and school

Key Findings from History

History of allergic rhinitis

Second network:
Increasing frequency of headaches
Vomiting/falling at soccer
Posterior location

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Stress at home and school

First network:
Normal neurological exam
No papilledema

Key Findings from Physical


Second network:
Left nystagmus

First network:
Tension headache
Stress reaction
Brain tumor

Differential Diagnosis

Second network:
Brain tumor
Basilar artery migraine

Brain MRI: Enhancing mass within

Key Findings from Testing

the left central portion of the

cerebellum and evidence of
obstructive hydrocephalus

Final Diagnosis

Cerebellar tumor

Case highlights: The students create an initial differential for the headaches, a
second differential for the new symptoms, and learn the diagnosis is a cerebellar
tumor. The case demonstrates how to deliver bad news to the patient and his
parents. Multimedia features include: MRI showing the tumor; photo of normal
optic nerve and fundus; photo of papilledema; video of a patient with nystagmus;
photo of a boy rubbing his nose in an allergic salute.

Key Teaching Points

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Brain tumors:
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
Risk factors
Exposure to ionizing radiation
Certain genetic syndromes, such as:
Tuberous sclerosis
Li-Fraumeni syndrome
Infratentorial lesions: Usually present with cerebellar signs and signs of
increased intracranial pressure
Cerebellar hemispheric lesions: May see changes in muscle tone and deep
tendon reflexes; more often find hypotonia and hyporeflexia
Supratentorial lesions: Focal motor and sensory abnormalities on side
opposite the lesion
Brain stem lesions: Often associated with cranial nerve and gaze palsies
Histologic types
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.
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 approximately 90% when completely
resected. Radiation reserved for those with high-grade tumors, partial
resections, or those in whom postoperative tumor progression is seen.
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 ranges from grave to good. Surgical resection alone
required for low-grade gliomas.
Ependymoma: Arise from within fourth ventricle (ependymal lining). Cause
symptoms related to hydrocephalus. Treatment usually surgical resection
plus radiation. Five-year survival approximately 50%.
Deaths from brain tumors are highest among all childhood cancer deaths.

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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
Tension headache:
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
Migraine headache:
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
Migraine types:
Classic: Accompanied by aura (visual symptoms, speech changes, or
other sensory abnormalities)
Common: Most frequent migraine type in children. No aura, frequently
unilateral (frontal or temporal)
Basilar artery: Uncommon migraine variant associated with bilateral
visual changes, paresthesias, and altered mental status
Migraine variants (seen exclusively in pediatric age group): Cyclical
vomiting, abdominal migraines, benign paroxysmal vertigo
Concerning symptoms
Headaches occur after a period of recumbency (e.g., early morning or after
a nap) or awaken patient from sleep
Headaches accompanied byand relieved afterforceful vomiting
Pain aggravating by bearing down (valsalva maneuvers)
Sudden onset
Headache accompanied by photophobia and fever
Headache accompanied by elevation in blood pressure, bradycardia, and
irregular respirations (Cushings triad)

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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 functions of the different parts of the cerebellum can help
localize lesions:
Lesions within the vermis (midline) cause dysarthria, truncal ataxia,
and gait abnormalities.
Cerebellar hemispheric lesions cause ipsilateral limb abnormalities,
nystagmus, tremor/dysmetria. Patients fall toward side of lesion and
have worse nystagmus when looking toward side of lesion.
Lesions of the deep cerebellar nuclei cause resting tremor, myoclonus,
and opsoclonus (such as seen in children with a neuroblastoma).
Ataxia: A term used to describe lack of coordination of muscle movements. This
sign may result from dysfunction in various parts of the nervous system, including
the cerebellum, the inner ear, and the dorsal columns. May be congenital or
acquired. A child with acute onset of ataxia requires immediate evaluation.
Post-infectious cerebellitis (acute cerebellar ataxia)
Believed to be an autoimmune response to viral illness (such as
varicella or coxsackie virus)
Causes cerebellar demyelination
Majority recover completely within a few months
Infectious cerebellitis
May be bacterial or viral in origin (mumps, enteroviruses, Epstein-Barr
virus, Streptococcus pneumonia, Neisseria meningitidis, Hemophilus
influenza B)
Fever and mental status changes often observed
Medication or toxin
Exposure to alcohol, antihistamines, or anti-convulsants
May be accompanied by nystagmus and dysmetria
Intracranial mass
Most often associated with tumors in the cerebellum or frontal lobe
Associated findings depend on area of involvement
Opsoclonus-myoclonus syndrome
Paraneoplastic syndrome that occurs most often with neuroblastoma
Ataxia is accompanied by intermittent jerking movements
(myoclonus) and erratic, jerky, conjugate movements of the eyes
Migraine headache
Basilar artery migraines or hemiplegic migraines may cause ataxia
Accompanying symptoms may include intermittent loss of vision,

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change in speech, headache, vomiting

Insidious onset, chronicincreasing loss of coordination over weeks to
Usually associated with headache and vomiting
Metabolic disease
Associated with several metabolic diseases, including maple syrup
urine disease and pyruvate decarboxylase deficiency
May be intermittent or chronic with intermittent exacerbations
Neurodegenerative disease
Ataxia telangiectasia and Friedrichs ataxia are most well known
Additional symptoms include loss of developmental milestones and
other neurological symptoms
Psychiatric illness
Conversion reaction can manifest as an hysterical involuntary gait

Headache: Eliciting a good history is crucial in evaluating a complaint of
headache. Remember to include:
Social history:
Headache may be due to anxiety, depression, or stressors in patients
Children may have somatic complaintsincluding headache that
have a psychological etiology.
Ask about childs home, family, school, and friends.
Headache history:
Character and duration of headaches
Associated symptoms
Activity at time of onset
Potential triggers and alleviating factors
Physical exam:
Neurological exam
Cranial nerves and visual fields:
Test visual acuity, extra-ocular muscles, 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,

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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:
The cerebellum helps to control balance and coordination. Lesions of
the cerebellum generally cause ipsilateral physical exam findings.
Romberg sign: Patient unable to maintain steady upright posture
while standing with arms extended anteriorly, palms upward, and
eyes closed or open
Test finger-to-nose, heel-to-shin, and rapid alternating movements
Plantar reflex:
Check Babinskis reflex (negative=downgoing)
Signs of allergies:
Allergic salute: Frequent upward rubbing of nose (salute) to alleviate itching
leads to transverse lines across lower third of nose
Dennies lines: Infraorbital transverse creases associated with chronic
conjunctival inflammation
Compassionate communication:
Prepare the environment in which bad news will be relayed.
Conversation should be conducted in a quiet, comfortable area in which
privacy is assured.
Ensure that the patients and/or parents support network is present.
The news should be delivered by one personthe most knowledgeable
person available.
Deliver the news slowly and clearly, taking time to answer questions fully to
maximize patients and/or parents understanding.
Sitting close enough to offer a touch when appropriate is helpful.
Be cognizant of ones body language and facial expressions, and that both
are consistent with the verbal message one is providing.

Differential diagnosis
Based on first clinic visit
1. Migraine: 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% of those
affected. The pattern is usually not progressive.
2. Tension headache: 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.

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3. Stress reaction: 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
4. Brain tumor: Likely to have focal neurological findings. Signs of increased
intracranial pressure, such as papilledema, are common with brain tumors,
but not always present. An increase in severity and frequency of headaches
is concerning.
5. Sinusitis: 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.
Based on second clinic visit
1. Brain tumor: Worsening headaches occurring in the morning associated
with emesis and ataxia point strongly to a progressive problem, such as
brain tumor.
2. Basilar migraine: 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.
3. Toxin/medication side effect: Ataxia is side effect of many medications.
Lateralizing signs on exam make a toxin less likely.

Magnetic resonance imaging (MRI): Provides excellent detail of the posterior
fossa. In children, frequently requires sedation.
Computed tomography (CT): Will not visualize the posterior fossa as well, but
is often easier and faster to obtain than MRI in some centers. May be valuable
when intracranial hemorrhage needs to be ruled out.
Lumbar puncture: Contraindicated in patient with signs of increased intracranial
pressure (may lead to brain herniation).

Daily headache diary: Asking patient or patients parent to keep a written log of
headachesincluding character and duration, location, associated symptoms,
activity at the time of the headache, potential triggers, and actions that relieved
the headachecan be a valuable clinical tool. The diary often includes more detail

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than can be recalled from memory alone.

Brain tumor:
Treatment: Multidisciplinary approach is key. Requires immediate involvement
Surgery will establish histologic diagnosis and reduce tumor burden.
Radiation therapy, chemotherapy, and other adjuvants (e.g., bone
marrow transplantation) may be indicated.
Social worker
Provides support and additional types of information
Primary care physician
Provides ongoing guidance and care throughout illness and in follow

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