Exam 3 - Neurology Flashcards by Kelsey Peiser

What is a non-progressive motor impairment secondary to fetal or infantile brain injury?

Cerebral palsy

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What is the most common subtype of cerebral palsy?

Spastic

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What are all the subtypes of cerebral palsy?

Spastic
Ataxic
Dyskinetic
Mixed

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The following signs/symptoms are concerning for what disorder?

Abnormal tone/posture
Retained primitive reflexes
Not reaching milestones
Excessive irritability
Poor feeding, drooling
Poor visual attention

Cerebral palsy

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What is the symptomatic management of cerebral palsy?

OT, PT, bracing
Anti-spasmodics
Botulism toxin (for contractures)

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The following signs/symptoms are concerning for what disorder?

Asymptomatic
Bradycardia, HTN, altered respiratory rate
Headaches, n/v, behavior changes, papilledema
Macrocephaly
Hyperreflexia, spasticity

Hydrocephalus

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How is Hydrocephalus diagnosed?

Newborns/infants - US

Older infants/children - MRI or CT

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What is the management for hydrocephalus?

Refer to neurosurgery for possible shunt placement

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When should occipitofrontal circumference (OFC) be measured?

Each well child visit b/w birth to 3 y/o

Any child w/ neurologic symptoms or developmental complaints

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How is microcephaly defined?

Head circumference 2 or more standard deviations below the mean or < 5th percentile

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What are the two types of microcephaly?

Primary (congenital) - lack of brain development or abnormal development due to timing of insult

Secondary (postnatal) - injury or insult to previously normal brain

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If symptomatic microcephaly, what might you expect to see in regards to possible signs/symptoms?

Delayed milestones
Seizures or spasticity
Fontanelle may close early and sutures may be prominent

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How is macrocephaly defined?

Head circumference 2 or more standard deviations above the mean or > 97th percentile

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What are the three types of growth patterns seen in macrocephaly and what do they suggest?

Rapid growth
- Increased ICP

Catch-up Growth
- Seen in premature infants, neurologically intact (under growth curve and then shoot up, catching up to normal growth)

Normal growth rate
- Familial macrocephaly or megaloencephaly

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What is the management for microcephaly and macrocephaly?

Neurology referral for labs and imaging

- Treat underlying cause

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What are the standard rates of growth in head circumference for the following age groups:
1-3 months
4-6 months
6-12 months

What would you do if a child’s measurement was above the average?

1-3 months: 2 cm/month
4-6 months: 1 cm/month
6-12 months: 0.5 cm/montn

1) Re-measure to ensure accuracy
2) If measurement is the same, consult neurology due to concern for increased ICP

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What is it called when cerebellar tonsils are abnormally shaped and displaced caudally below the foramen magnum?

Chiari Malformation - Type I

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When do symptoms of Chiari Malformation - Type I typically present?

Not until teen or adult

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What is a syringomyelia and what can it be associated with?

Fluid filled cyst within the spinal cord

Can be associated with Chiari Malformation - Type I

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What are signs/symptoms associated with Chiari Malformation - Type I?

Asymptomatic
Neck pain, headaches
Increased ICP
UMN symptoms (hyperreflexia, weakness)
Sleep apnea
Worse with Valsalva, coughing, sneezing
Syringomyelia symptoms

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What are signs/symptoms associated with a syringomyelia?

Cape-like sensory loss

- Loss of abdominal reflex

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What is a Chiari Malformation - Type II?

Type I + myelomeningocele

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When is Chiari Malformation - Type II typically detected?

Prenatally or at birth

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What are signs/symptoms associated with Chiari Malformation - Type II?

Dysphagia
Apneic spells and aspiration
UE weakness

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How are Chiari Malformations diagnosed if not at birth?

MRI (tonsils are 5 or more mm below foramen magnum)

What is the management for Chiari Malformations?

- Neurosurgery consultation - Asymptomatic Type I - conservative - Type II - surgical

What are etiologies/risk factors for spina bifida?

- Low folate - Genetics - Fever/hyperthermia in 1st trimester - Poorly managed diabetes - Obesity

What are the type of spinal bifida?

Closed = Spina bifida occulta Open = Meningocele, Myelomeningocele

What is Closed Spinal Dysraphism/Spina Bifida Occulta?

Incomplete closure of the spinal cord

What is the presentation of Closed Spinal Dysraphism/Spina Bifida Occulta?

Can range from asymptomatic to severe abnormalities. Hairy patch, dimple, dark spot, swelling on the back at the site of the gap in the spine.

What is Open Spinal Dysraphism/Meningocele?

Outpouching of the spinal fluid and meninges through the vertebral cleft.

What is Open Spinal Dysraphism/Myelomeningocele?

Meninges and spinal cord protrude from the vertebral cleft.

What symptoms are associated with Open Spinal Dysraphism/Myelomeningocele?

- Sensory loss - Paralysis - Loss of bladder/bowel control

What is associated with Open Spinal Dysraphism/Myelomeningocele in a majority of cases?

Chiari II malformation and hydrocephalus

How is Spina Bifida diagnosed?

MRI

The following clinical manifestations are associated with what disorder? - Cafe-au-lait macules - Neurofibromas - Axillary/inguinal freckling - Lisch nodules - Optic pathway glioma

Neurofibromatosis (NF1)

What are neurological abnormalities associated with Neurofibromatosis (NF1)?

Macrocephaly, seizures, cognitive deficits

What is the management for Neurofibromatosis (NF1)?

Multi-disciplinary team of neurology, genetics, and ophthalmology

What is the diagnostic criteria for Neurofibromatosis (NF1)?

Two or more of the following clinical features must be present: - Six or more cafe-au-lait macules - Two or more neurofibromas - Axillary/inguinal freckling - Optic glioma - Two or more Lisch nodules - Distinctive bony lesion - First-degree relative with NF1

What are the most common types of primary headaches?

Migraine and tension

What are the most common types of secondary headaches?

Acute febrile illness

The following description is associated with which type of headache? - Location - focal - Duration - 2-72 hours - Character - moderate to severe, pulsatile/throbbing - Aggravated with activity - Associated N/V, photo/phonophobia, +/- aura

Migraine (primary)

The following description is associated with which type of headache? - Location - diffuse - Duration - 30 min to 7 days - Character - mild to moderate, pressure/non-throbbing - Not aggravated with activity - May have photo or phonophobia, but not both

Tension (primary)

How are headaches managed/prevented?

- Track/avoid triggers - Exercise, hydration, meal schedule - Symptomatic treatment w/ ibuprofen, Tylenol - Relaxation therapy, decrease screen time

What are some worrisome headache features and when should you considering referring the patient?

- Abnormal neurologic or visual exam - Severe headache upon awakening/awaken in middle of night - Daily symptoms with progressive worsening - Accompanied with persistent vomiting - Acute onset without previous history - Increased with coughing or bending

In what population is pseudotumor cerebri typically seen?

Obese teenage girls

What is the hallmark symptom of pseudotumor cerebri?

Papilledema

What are the following signs/symptoms associated with? - Headache - Papilledema - Visual symptoms - Pulsatile tinnitus - Neck stiffness/back pain

Pseudotumor Cerebri

How is Pseudotumor Cerebri diagnosed?

Diagnosis of exclusion Neurological evaluation - MRI - LP Ophthalmologic evaluation

What is the management for Pseudotumor Cerebri?

- Acetazolamide (reduce rate of CSF production) - Topiramate (help control HA) - Weight loss

What is a sudden, transient disturbance of brain function manifested by involuntary sensory, motor, autonomic symptoms with or without loss of consciousness?

Seizure

Define epilepsy.

2 or more unprovoked seizures occuring more than 24 hours apart

What are the phases of a seizure?

- Aura: sense of physical or emotional warning of imminent seizure - Ictal: active seizure - Post-ictal: recovery period

What are focal seizures?

Seizures occurring in localized region of the brain

Describe the presentation of a simple focal seizure.

Conscious: motor, sensory, autonomic symptoms lasting seconds to minutes

Describe the presentation of a complex focal seizure.

Impaired awareness: interruption of behavior or staring, yelling, arm movements lasting < 3 minutes

What are generalized seizures?

Abnormal electrical charges in both hemispheres simultaneously

Describe the varying types of seizures below: - Absence - Myoclonic - Tonic - Clonic - Atonic

All are with loss of consciousness or awareness and varying post-ictal state Absence = blank stare, automatisms Myoclonic = sudden, brief muscle contractions Tonic = rigid Clonic = rhythmic jerking Atonic = sudden loss of muscle control

What is the most common type of generalized seizure?

Tonic/clonic = Rigid/Rhythmic jerking

The following clinical features are associated with what type of seizure? - Sudden impairment of consciousness w/o loss of tone - Provoked by hyperventilation - Presents b/w age 4-10 and often remits by puberty - Lasts 9-10 seconds and may occur 10 times/day

Absence Seizures

How are absence seizures diagnosed?

History, exam, EEG

What is the 1st line medication for absence seizures?

Ethosuximide

What are absence seizures provoked by?

Hyperventilation

What are clinical features associated with febrile seizures?

- Convulsion with temp > 38C (100.4F) - Age 6 months to 5 years - Often associated with virus - (+) genetic predisposition

Compare a simple febrile seizure to a complex febrile seizure.

Simple - most common, lasting < 15 minutes Complex - focal (one side of body), lasting > 15 minutes or occurs > 1 in 24 hours

What is the management for febrile seizures?

- Generally self-limiting | - IV benzodiazepines if symptoms lasting > 5 minutes

What is an acute immune-mediated polyneuropathy that is typically preceded by illness, most commonly Campylobacter jejuni?

Guillian-Barre Syndrome

What is the most common cause of acute flaccid paralysis in healthy infant/child?

Guillian-Barre Syndrome

The following signs/symptoms are associated with what disorder? - Ascending symmetric weakness - Neuropathic pain - Gait instability or refusal to walk - Absent reflexes

Guillian-Barre Syndrome

How is Guillian-Barre Syndrome diagnosed?

- Electrodiagnostic studies (EMG/NCV) - CSF analysis (increased protein with normal WBC) - Spinal MRI with and w/o contrast

What is the management of Guillian-Barre Syndrome?

- Hospitalization and close monitoring if rapidly progressing - IVIG or plasma exchange in severe cases

The following signs/symptoms are associated with what disorder? - Descending weakness - Constipation, poor feeding - Hypotonia, loss of DTR's - Irritable and lethargic

Botulism

What diagnostic studies are used to evaluate for botulism?

- Stool sample | - EMG/NCV

What is the management for Botulism?

Hospitalization and close monitoring | - Botulism immune globulin

In Duchenne Muscular Dystrophy, what may be elevated before onset of symptoms?

Elevated muscle enzymes (CK > 10-20 times normal)

What specific sign is associated with Duchenne Muscular Dystrophy?

Gower's sign (use hands to push up from floor)

The following signs/symptoms are associated with what disorder? - Progressive weakness (proximal before distal, UE before LE) - Gower's sign - Pseudohypertrophy of calves

Duchenne Muscular Dystrophy

What are some conditions associated with Duchenne Muscular Dystrophy?

- Cardiomyopathy - Orthopedic complications (scoliosis, fractures) - Growth delay - Cognitive impairment

What diagnostic studies can be used to evaluate for Duchenne Muscular Dystrophy?

- Elevated muscle enzymes (CK > than 10-20 times normal) | - Genetic testing

What is the management of Duchenne Muscular Dystrophy?

Glucocorticoids

What is the typical age of onset in Duchenne Muscular Dystrophy?

Age 2-3 (earlier onset then Becker's)

How does Becker Muscular Dystrophy differ from Duchenne Muscular Dystrophy?

- Symptom onset is later - Muscle involvement not as severe - CK elevated > 5 times normal, not > 10-20 - Cardiomyopathy may be more predominant

Is Guillian-Barre Syndrome associated with descending or ascending weakness?

Ascending

Is Botulism associated with descending or ascending weakness?

Descending