20 Flashcards
Tension HA
Tension headaches often occur in the setting of emotional stress (at home, school or related to peer interactions), fatigue, lack of sleep, and other stressors.
This type of headache typically:
is most often episodic
generally worsens throughout the day
is of mild to moderate intensity
may feel like a band around the head or involve the occipital area with accompanying tenderness of the posterior muscles of the neck
Migraine HA in children Migraine variants 3 Sxs Precipitating factors 4 RF alleviating factors Classic vs common migraine HA
Migraine headaches are the most common cause of recurrent headache in children.
It is also worth mentioning that although uncommon, migraines can first manifest as periodic syndromes:
cyclical vomiting
abdominal migraines
benign paroxysmal vertigo
These atypical “migraine variants” are seen exclusively in the pediatric age group.
Symptoms
more severe than tension headaches
often of a throbbing nature
often accompanied by photophobia and/or phonophobia, abdominal pain, nausea, and vomiting
Precipitating Factors
Common precipitating factors of migraines:
stress bright lights odors (such as perfumes) foods Alleviating Factors
frequently relieved by sleep
Risk Factors
Family history is positive for migraine headaches in a majority of those affected.
“Classic” vs. “Common”
A “classic” migraine headache is one that is accompanied by an “aura,” which can include visual symptoms (bright spots in the visual field), speech changes or sensory abnormalities (such as paresthesias).
A “common” migraine headache—also referred to as migraine without an aura—is the most frequent type of migrainous headache seen in children; it is typically unilateral, frontal or temporal in location, but may involve any part of the head.
Concerns for life threatening causes of HA in children 6
Occurring with forceful vomiting after lying down Headaches that 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 should raise concern for increased intracranial pressure ICP, which can be life-threatening.
Sudden onset 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.
Awakens from sleep May also be a sign of increased ICP.
Accompanied by fever and photophobia 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.
Worsens with cough or Valsava Headache that is aggravated when the patient performs maneuvers such as bearing down to have a bowel movement, coughing (or the Valsalva maneuver), or bending over are concerning for increased ICP.
Progressively worsening Headaches that are progressively increasing in frequency and/or severity are of greater concern for a more serious pathologic process,
Children and stress- how they present 3
Children who are exposed to a stressful environment may present to the pediatrician with somatic complaints, such as abdominal pain, chest pain, or headaches.
Vital signs that warrant a life threatening cause for HA 5
Recognizing abnormalities in vitals signs in children of different ages is critical in any clinical setting:
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.
Elevations in blood pressure, along with bradycardia and irregular respirations (Cushing’s triad) are suggestive of increased intracranial pressure and would warrant immediate institution of life-saving interventions.
Romberg test
The Romberg test is performed by asking the patient to stand with feet together and arms extended anteriorly, palms upward.
The ability to maintain an upright posture is noted with eyes opened and then with eyes closed. Minimal swaying should occur.
With diseases resulting in decreased position sense or abnormal vestibular function, the Romberg test will be + only with eyes closed; vision helps the patient compensate for the abnormality.
In patients with cerebellar pathology, the Romberg test is abnormal (+) with eyes opened and eyes closed.
Idiopathic intracranial HTN pathophys Sxs Etiology Dx Complications
Pathophysiology
Idiopathic Intracranial Hypertension refers to the condition of increased intracranial pressure in the absence of intracranial mass lesion or hydrocephalus, and it often mimics brain tumors in presentation. It is thought to occur because of alterations in CSF absorption and/or production, or abnormalities in vasomotor control and cerebral blood flow.
Symptoms
Headache is the most common presenting complaint.
Vomiting may occur.
Diplopia secondary to paralysis of the abducens nerve may be present.
Etiology
Idiopathic Intracranial Hypertension has been associated with many causal/predisposing factors, including:
Obesity
Medications (e.g., vitamin A derivatives, tetracycline, oral contraceptives, steroids)
Metabolic disorders (e.g., galactosemia, hypoparathyroidism)
Infections (e.g., sinusitis, chronic otitis media)
Diagnosis
Brain imaging studies are usually normal. In some children the ventricles may be small. No mass lesion is seen.
Overall there are 3 major diagnostic criteria:
Increased intracranial pressure (with associated symptoms such as headache and vomiting)
Normal brain anatomy
Normal cerebrospinal fluid
The diagnostic test is an elevated CSF opening pressure during a lumbar puncture.
Complications
The main concerning long-term sequela of delay in recognizing and treating Idiopathic Intracranial Hypertension is blindness resulting from transmission of elevated CSF pressure to the optic nerve sheath.
When to use a HA diary
When more information is needed to clarify the cause of headache, a daily diary including the following can be very valuable:
Character of headache Duration Location Associated symptoms Activity at the time of the headache Potential triggers Actions that relieved the headache (alleviating factors) The diary often provides more detail than can be recalled from memory alone.
Ataxia
Diff dx
Ataxia (from the Greek ataxos meaning “lack of order”) is a term used to describe lack of coordination of muscle movements.
Ataxia is a non-specific neurologic sign which can result from dysfunction in various parts of the nervous system that coordinate movement, such as the cerebellum, the inner ear, and the dorsal columns.
Ataxia can be congenital or acquired.
Acute ataxia is a frequent presentation to primary care providers, emergency departments and neurologists. Due to the varied differential diagnoses, some of which can be life-threatening, immediate evaluation of a child with sudden onset of ataxia is important.
Each of the following is in the differential diagnosis of acquired ataxia:
Post-infectious cerebellitis
Post-infectious cerebellitis (otherwise known as acute cerebellar ataxia) is the most common cause of acute ataxia in children.
Primarily a diagnosis of exclusion in children 1 to 3 years of age.
Thought to be an autoimmune response leading to cerebellar demyelination.
Occurs several weeks after a viral infection (e.g., varicella or coxsackie virus).
Onset is sudden and consists of ataxia, vomiting, nystagmus in about half of the patients and dysarthria in some.
CSF may be normal or have a pleocytosis; eventually the CSF protein is elevated.
Majority of children recover completely within a few months.
Not typically associated with fever or other systemic manifestations.
Infectious cerebellitis
May be viral or bacterial in etiology.
Fever is often an accompanying symptom.
Mental status changes are often observed.
Examples of pathogens include mumps, enteroviruses and Epstein-Barr virus.
Bacterial pathogens include those that cause bacterial meningitis (e.g., Streptococcus pneumoniae, Neisseria meningitidis and Haemophilus influenzae type B.
Medication or toxin
Exposure to a medication such as an anticonvulsant, or to a toxin such as alcohol (or even to sedating antihistamines) can lead to acute ataxia.
The 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.
Intracranial mass
An intracranial mass may lead to 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.
Opsoclonus-myoclonus syndrome
A paraneoplastic syndrome that occurs most often with neuroblastoma, generally occurs in the younger child (6 months-3 years).
Ataxia is accompanied by intermittent jerking movements (myoclonus) and erratic, jerky conjugate movements of the eyes (opsoclonus).
Migraine headache
Basilar artery migraines or hemiplegic migraines can cause recurrent intermittent episodes of acute ataxia.
Accompanying symptoms may include intermittent loss of vision, change in speech, headache and vomiting.
Hydrocephalus
Ataxia associated with hydrocephalus generally is insidious in onset and quite chronic with increasing loss of coordination over weeks to months.
It is usually associated with headache and vomiting.
Metabolic disease
Ataxia may be associated with a number of metabolic diseases such as maple syrup urine disease or pyruvate decarboxylase deficiency.
Here the ataxia may be intermittent or chronic with intermittent exacerbations.
Neurodegenerative disease
There are several neurodegenerative diseases of childhood that present with ataxia: 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.
Psychiatric illness
A psychiatric disorder known as conversion reaction can manifest as a 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.
The child is generally able to sit without difficulty, but when put in a standing position, immediately begins to sway at the waist.
Basilar migraine
Basilar migraine is an uncommon migraine variant. Patients, 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. There may be associated:
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. The impairment in consciousness, in turn, is followed by a throbbing headache, which is usually occipital, often with nausea and vomiting.
Since the 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.”
Nystagmus
Sign of what diseased part of the brain
A rhythmic oscillation of the eyes, analogous to tremor in other parts of the body.
May occur in one or more planes (horizontal, vertical or rotary).
Usually quicker in one direction than in the other and is defined by its quicker phase.
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.
Cerebellum
Function
Lesion location and the sxs associated with that location
The cerebellum (Latin for “little brain”) plays an important role in motor control. While it does not initiate movements, it is responsible for coordination, precision, and controlling balance. Due to the close proximity of the cerebellum to the 4th ventricle, mass lesions in the cerebellum often lead to obstructive hydrocephalus (see Figure 1).
Different parts of the cerebellum have different functions, knowledge of which can help localize lesions within the cerebellum ( Figure 2).
Lesions within the 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.
Visualization of suspected brain tumor - MRI vs CT
Magnetic resonance imaging (MRI) would provide excellent detail of the posterior fossa.
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.
Distinguishing neurological exam findings - infratentorial, supratentorial, cerebellar, brain stem
Infratentorial lesions usually present with cerebellar signs and signs of raised intracranial pressure (ICP).
Cerebellar hemispheric lesions can cause changes in muscle tone and DTRs, but usually lead to hypotonia and hyporeflexia.
Supratentorial lesions more commonly lead to focal motor and sensory abnormalities on the side opposite to the lesion.
Brain stem tumors will often be associated with cranial nerve and gaze palsies.
Brain tumors
Incidence of age
RF
Classifications/types - “GAME”
Incidence by Age
Brain tumors are more common in the younger age group (31 cases /million children annually for those < 7 years of age versus 26 cases/million children for the older age group).
The incidence peaks in the first decade of life, with a second peak in older adulthood (8th decade).
Males are slightly overrepresented in the childhood brain tumor population, but this 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 first 2 years of age; for the rest of the first decade, infratentorial tumors predominate.
In adolescence and adulthood, supratentorial tumors once again become more common.
Risk Factors
The only two risk factors that have been consistently identified for childhood brain tumors are:
Exposure to ionizing radiation, and
Certain genetic syndromes (e.g., tuberous sclerosis, neurofibromatosis, and the very rare Li-Fraumeni syndrome).
Classification
Overall, childhood primary brain tumors are classified according to histology. Histologically, the most common tumors are:
Medulloblastoma (20%)
Juvenile pilocytic astrocytoma (20%)
Low-grade astrocytoma (15%)
High-grade astrocytoma (7%)
Brain stem gliomas
Brain stem gliomas 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.
Surgical resection alone is required for low-grade gliomas.
Prognosis ranges from quite grave to quite good
Ependymomas
Ependymomas arise from within the fourth ventricle (from the ependymal lining) and cause symptoms related to hydrocephalus.
Treatment generally includes surgical resection, followed by radiation.
5-year survival is approximately 50%.
Astrocytoma of the cerebellum
Astrocytoma of the cerebellum has the best prognosis of all infratentorial tumors in children.
These often have a cystic component.
Treatment is surgical 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.
5-year survival is approximately 90% with complete resection.
Survival rates drop to 50-70% with partial resection.
Medulloblastoma
Medulloblastoma or infratentorial primitive neuroectodermal tumor is the most common pediatric brain tumor.
It is a malignant tumor that may spread throughout the central nervous system and is capable of metastasizing to extracranial sites.
Treatment generally includes surgical resection, radiation, and chemotherapy.
Prognosis is dependent on the size and dissemination of the tumor.
