Peds Case 20- 7Y/o W Headache

Front 1

tension headache

Back 1

episodic,
worsen throughout the day
mild to moderate intensity
feel like a band around the head or involve the occipital area with accompanying tenderness of the posterior muscles of the neck.
emotional stress (at home, school or related to peer interactions), fatigue, lack of sleep, and other stressors.

Front 2

migraine

Back 2

most common cause of recurrent headache in children
more severe and often of a throbbing nature (contrast to tension)
precipitating factors include stress, bright lights, odors (such as perfumes) and foods.
relieved by sleep.
Family history
accompanied by an ‘aura,’ which can include visual symptoms (bright spots in the visual field), speech changes or sensory abnormalities (such as paresthesias).
"common" migraine headache, migraine without an aura, is the most frequent type of migrainous headache seen in children.
unilateral, frontal or temporal in location, but may involve any part of the head.
Photophobia and/or phonophobia, abdominal pain, nausea and vomiting
can first manifest as periodic syndromes-cyclical vomiting, abdominal migraines and benign paroxysmal vertigo= atypical ‘migraine variants’ exclusively in the pediatric age group

Front 3

worrisome features of headaches

Back 3

occur after a period of recumbency (e.g., early morning or after a nap) and that may be accompanied by, and relieved after, forceful vomiting= increased intracranial pressure,
may awaken the patient from sleep
aggravated when maneuvers increase intracranial pressure, bearing down to have a bowel movement, coughing (or the valsalva maneuver), or bending over.

Although sudden onset of severe headaches may be seen in children with migraines, this may also be a sign of a more serious process such as intracranial hemorrhage.
Although migraine headaches can be accompanied by photophobia, headache in a child with photophobia and fever, should prompt investigation for an infectious etiology such as meningitis or encephalitis.

Headaches that are progressively increasing in frequency and/or severity are of greater concern for a more serious pathologic process, in contrast to intermittent headaches that have persisted without change for a prolonged period of time.

Front 4

stressful environment may present to the pediatrician with somatic, rather than psychological or mental complaints.

Back 4

These somatic complaints may include abdominal pain, chest pain or headaches.

Front 5

development and migranes

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Abnormal development would cause the clinician to consider a potential relationship between the developmental delay and the headaches. In the child who has always been delayed, a link between the cause of headaches and the delay may be unlikely. Loss of developmental milestones, however, would be very concerning and would cause one to consider a neurologic process accounting for both the headaches and developmental change

Front 6

genetics and migranes

Back 6

genetic basis but no genes identified

Familial Hemiplegic Migraine or FHM- rare; calcium channel gene mutations and alterations in the gene for a sodium/potassium ATPase.
single nucleotide polymorphisms in the more common types of migraines

A single nucleotide polymorphism ("SNP") is the change of one base in a gene. Technically, a specific change needs to be present in at least 1% of the population to be classified as a polymorphism.

Sometimes, the nucleotide change won't affect the final amino acid sequence because the genetic code is redundant= silent mutation.
change a single amino acid, which may or may not have serious effects= missense mutation.
introduce a "stop codon,"= nonsense mutation.

Front 7

concerning vitals in headaches

Back 7

Fever and tachycardia in a child with headache may indicate a serious infectious process such as meningitis, encephalitis, intracranial abscess (such as from complications of sinusitis or otitis media), or even sepsis

cushing's triad
Elevations in blood pressure, along with bradycardia and irregular respirations = increased intracranial pressure

Front 8

Dennie's lines

Back 8

infraorbital transverse creases
mild crhonic inflamm of conjunctivae in allergic conjunctivitis

Front 9

cobblestoning in eyes

Back 9

cobblestone-like giant papillae of the opper tarsal conjunctiva

Front 10

pseudotumor cerebri

Back 10

increased intracranial pressure in the absence of intracranial mass lesion or hydrocephalus
often mimics brain tumors in presentation.
alterations in CSF absorption and/or production, or abnormalities in vasomotor control and cerebral blood flow.
Headache most common presenting complaint. Vomiting may occur. Diplopia secondary to paralysis of the abducens nerve may be present.

causal/predisposing factors: obesity, medications (e.g., vitamin A derivatives, tetracycline, oral contraceptives, steroids), metabolic disorders (e.g., galactosemia, hypoparathyroidism) and infections (e.g., sinusitis, chronic otitis media).

Brain imaging studies nml
ventricles may be small. No mass lesion is seen

3 major diagnostic criteria:
increased intracranial pressure (with associated symptoms such as headache and vomiting),
normal brain anatomy,
normal cerebrospinal fluid.

dx: elevated CSF opening pressure during a lumbar puncture.

blindness resulting from transmission of elevated CSF pressure to the optic nerve sheath

Front 11

ddx headaches

Back 11

long-standing nature of Nick’s headaches makes a brain tumor less likely as a cause of the chronic headaches, the recent increase in his headaches is worrisome and makes this a diagnosis worth keeping in consideration.

Poorly controlled allergic rhinitis can trigger exacerbations of acute and chronic sinusitis, common causes of headaches in children.

A family history of migraine is present in about 50%of those with migraine headaches

Stress can trigger migraine headaches and also tension headaches. Often other somatic complaints are also seen such as difficulty sleeping, deteriorating school performance and lack of interest in interacting and playing with friends.

Children with a brain tumor often have focal neurologic abnormalities or signs of increased intracranial pressure.

Front 12

ataxia

Back 12

lack of coordination in muscle mvmts (nonspecific)
cerebellum, the inner ear and the dorsal columns.

congenital or acquired.

ddx:
Post-infectious cerebellitis
Infectious cerebellitis
Medication or toxin
Intracranial mass
Opsoclonus-myoclonus syndrome
Migraine headache
Hydrocephalus
Metabolic disease
Neurodegenerative disease
Psychiatric illness

Sinusitis, tension headache and stress would not cause ataxia.

Front 13

Post-infectious cerebellitis, acute cerebellar ataxia,

most common cause of acute cerebellar ataxia in children

Back 13

diagnosis of exclusion 1 to 3 y/o
autoimmune response --> cerebellar demyelination, several weeks after a viral infection
varicella or coxsackie virus.
sudden ataxia, vomiting, nystagmus in about half; dysarthria in some.
CSF nml or pleocytosis; eventually the CSF protein is elevated
majority recover completely within few months.
pure form, is not associated with fever or other systemic manifestations.

Front 14

Acute infectious cerebellitis

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viral or bacterial in etiology
Fever is often an accompanying symptom, and mental status changes are often observed.

mumps, enteroviruses and Epstein-Barr virus.
Bacterial: (those that cause bacterial meningitis) Streptococcus pneumoniae, Neisseria meningitidis and Hemophilus influenzae B.

Front 15

medications or toxin (ataxia)

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anticonvulsant, or to a toxin such as alcohol (or even to sedating anthistamines) can lead to acute ataxia.

may be accompanied by nystagmus and dysmetria and is usually bilateral, owing to diffuse involvement of the cerebellum including the vermis and the cerebellar hemispheres.

Front 16

intracranial mass

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ataxia that is acute or chronic, depending on how early the lesion is identified and the extent of involvement.

Ataxia is most often associated with tumors in the cerebellum or frontal lobe. Associated findings depend on the precise area of involvement.

Front 17

Opsoclonus-myoclonus syndrome

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paraneoplastic syndrome that occurs most often with neuroblastoma,
younger child (6 months-3 years). P
ataxia is accompanied by intermittent jerking movements (myoclonus) and erratic, jerky conjugate movements of the eyes (opsoclonus)

Front 18

Basilar artery migraines or hemiplegic migraines

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recurrent intermittent episodes of acute ataxia. Accompanying symptoms may include intermittent loss of vision, change in speech, headache and vomiting.

uncommon migraine variant.
usually young women or children, first develop visual phenomena like those of typical migraine except that they are bilateral; transient cortical blindness may also occur.
vertigo, ataxia, incoordination of the limbs, dysarthria, and bilateral limb and perioral paresthesias. This is sometimes followed by transient loss or impairment of consciousness or by a confusional state
followed by a throbbing headache, which is usually occipital, often with nausea and vomiting.
symptoms resemble those caused by ischemia in the territory of the basilar-posterior cerebral arteries, this entity is referred to as basilar migraine or basilar artery migraine.

Front 19

hydrocephalus

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ataxia insidious in onset and quite chronic with increasing loss of coordination over weeks to months
associated with headache and vomiting.

Front 20

metabolic diseases

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maple syrup urine disease or pyruvate decarboxylase deficiency. Here the ataxia may be intermittent or chronic with intermittent exacerbations.

Front 21

neurodegenerative

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ataxia-telangiectasia and Friedrich ataxia are the most well known.
Most affected children are younger than 10, and their symptoms include a loss of developmental milestones, ataxia and other neurological symptoms.

Front 22

psych conversion rxn

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hysterical involuntary disturbance in gait (patient truly believes something is wrong, but no physical pathology exists) known as astasia-abasia.

Unlike true ataxia, this gait is wildly erratic and involves lurching of the body which requires extraordinary balance.
able to sit without difficulty, but when put in a standing position, immediately begins to sway at the waist.

can manifest as ataxia (or pseudoataxia), patients with conversion disorder rarely fall.

Front 23

Nystagmus

Back 23

rhythmic oscillation of the eyes, analogous to tremor in other parts of the body. Nystagmus may occur in one or more planes (horizontal, vertical or rotary). Nystagmus is usually quicker in one direction than in the other and is defined by its quicker phase. Nystagmus is a sign of cerebellar disease, and has many possible causes including tumors, toxins, cerebellitis and multiple sclerosis. When nystagmus occurs because of cerebellar disease, it is most prominent when the patient is looking towards the side of the lesion.

Front 24

cerebellum

Back 24

does not initiate movements, it is responsible for coordination, precision, and controlling balance
mass lesions in the cerebellum often lead to obstructive hydrocephalus b/c close to 4th ventricle

vermis (midline) cause dysarthria, truncal ataxia, and gait abnormalities.
Cerebellar hemispheric lesions cause ipsilateral limb abnormalities, nystagmus, tremor/dysmetria and tend to spare speech.

Patients fall towards the side of the lesion and have worse nystagmus when they look towards the side of the lesion.

Lesions of the deep cerebellar nuclei cause resting tremor, myoclonus, and opsoclonus such as that seen in children with a neuroblastoma.

Nick is ataxic and falls to the left side. He is not dysarthric. He has left-sided past-pointing, and has nystagmus that is most pronounced on left lateral gaze. These signs, along with the left leg hypotonia and hyporeflexia indicate that the cerebellar lesion is in the left cerebellar hemisphere. You are concerned that the tumor could be causing obstructive hydrocephalus (based on the presence of headache, emesis and papilledema).

Front 25

test for cerebellar lesion

Back 25

Computed tomography (CT) is not good in visualizing the posterior fossa quite as well. In cases when intracranial hemorrhage is suspected or needs to be ruled out, a head CT may be valuable as the first test since it is often easier and faster to obtain than an MRI in some centers, and unlike MRI rarely requires sedation of the child. A lumbar puncture is contraindicated in a patient with increased intracranial pressure and focal findings on neurologic exam, and may lead to brain herniation.

Beta human chorionic gonadotropin and alpha-fetoprotein are produced by germ cell tumors, which are most often located in the pineal gland or suprasellar region. Tumors in those areas often present with headache, but also with visual defects and/or cranial nerve defects. Nick's vision exam is normal and his neurological exam does not reveal

Front 26

brain tumors

Back 26

most common solid tumor in children and are the second most common form of childhood cancer behind leukemia; the incidence of brain tumors has been increasing over the past several decades; the reasons behind this are not entirely clear. Although the mortality rate with brain tumors has decreased by 20% in children since 1975, this improvement in survival is disproportionately less than that seen with other childhood cancers. Deaths caused by childhood brain tumors are the highest among all childhood cancer deaths. Moreover, the morbidity associated with brain tumors is significant and includes motor, neurocognitive and neuroendocrine sequelae.

more common in the younger age group
male predominance can be accounted for by the higher male incidence of only two types of brain tumors-ependymomas and medulloblastomas

Supratentorial tumors are more common in the 1st 2 years of age. For the rest of the 1st decade, infratentorial tumors predominate. In adolescence and adulthood, supratentorial tumors once again become more common.

only prediposition: ionizing radiation and certain genetic syndromes such as tuberous sclerosis, neurofibromatosis, and the very rare Li-Fraumeni syndrome.

histologically, the most common tumors are: medulloblastoma (20%), juvenile pilocytic astrocytoma (20%), low-grade astrocytoma (15%) and high-grade astrocytoma (7%).

Complications of the brain tumor or of therapy may include obstructive hydrocephalus, seizures and hypothalamic-pituitary hormonal insufficiency.

Front 27

Brain stem gliomas

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may be either quite aggressive, resulting in diffuse infiltration of the pons, or may be low-grade in nature, resulting in a focal tumor in the midbrain or medulla. Prognosis, therefore, ranges from quite grave to quite good, with surgical resection alone required for low-grade gliomas.



has the

is the

Front 28

Ependymomas

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from within the fourth ventricle (from the ependymal lining) and cause symptoms related to hydrocephalus. Treatment generally includes surgical resection, followed by radiation, and carries a 5-year survival of approximately 50%.

Front 29

Astrocytoma of the cerebellum

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best prognosis of all infratentorial tumors in children. often have cystic component.

Treatment is surgical resection; the 5-year survival is approximately 90% with complete resection. Survival rates drop to 50-70% with partial resection. Radiation therapy is reserved for patients with high-grade astrocytomas, partial resections, or those patients in whom postoperative tumor progression is evident by clinical and radiologic investigation.

Front 30

Medulloblastoma or infratentorial primitive neuroectodermal tumor

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most common pediatric brain tumor.
malignant tumor that may spread throughout the central nervous system and is capable of metastasizing to extracranial sites. The prognosis and treatment are dependent on the size and dissemination of the tumor. Treatment generally includes surgical resection, radiation and chemotherapy.

Front 31

long-term sequelae of childhood brain tumors are most often from the effects of

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chemotherapy and/or radiation therapy. The most common are neurocognitive defects, attention deficit disorder, learning disabilities, endocrine abnormalities and stroke. The rate of overall IQ deficits is associated with several risk factors: younger age at time of treatment, longer time elapsed since treatment, female gender, hydrocephalus, use of radiation therapy and its extent and dose..