Neuro Flashcards
Gower sign is?
Arises from floor by pulling on legs and body to get up
Pronator drift is?
Weaker hand - cups and supinates
Muscle fasciculations are?
Denervation from anterior horn cell or peripheral nerve disease.
LMN and Cerebellar lesions will affect tone how?
Hypotonia
UMN lesions will affect tone how?
Hypertonia - Spasticity
Extrapyramidal disease affects tone how?
Rigidity - resistance through ROM
Dysfx of cerebellar pathways will affect coordination how?
Ataxia, Intention tremor, Dysmetria (cant judge distance)
Cerebellar dysx affects gait how?
Broad-based, unsteady gait, that cant turn
Corticospinal tract dysfx affects gait how?
Stiff, scissoring gait and TOE walking
Extrapyramidal dysfx affects gait how?
Slow, Stiff, SHUFFLING gait w/ dystonic postures
LMN or neuromuscular d/o affects gait how?
Waddling gait w/ hip weakness.
LMN dz affects DTRs how?
Decreased DTRs
UMN dz affects DTRs how?
Increased DTRs
Corticospinal tract dysfx affects DTRs how?
Babinski response OR extensor plantar reflex
REDFLAGS of HAs?
Worst HA of my life - SAH AM HA - Tumor Pain wakes child at night Chronic progressive HA (Most ominous) ABD/Focal neuro exam Changes w/ positioning - Pseudotumor cerebri Recurrent vomiting No family Hx
Worst HA of my life may indicate?
SAH
AM HA may indicate?
Tumor
Most ominous HA pattern?
Chronic progression
REDFLAGs of HA that overlap w/ migraines?
Changes w/ position
Recurrent vomiting
MC recurrent HA pattern?
Tension HA
Tension HA attributes?
Mild - lack S/S - Not disruptive
PAIN = global, squeezing/pressing
LASTS - Hrs to days
W/OUT - N/V, phonophobia/photophobia
Migraine HA attributes?
Frontal, Bitemporal, Unilateral PAIN - Mod/sev = Pounding/throbbing LASTS - 1-72hr Aggravated by activity and has AURA (15-30m) W/ - N/V, Photo/Phono-phobia, pallor Wants to seek dark quiet room to rest
Auras are experienced as?
Visual - Spots, flashes, flickering lines of light
Atypical - Perioral numbness, vertigo, unilateral weak
2ndy reasons for HAs?
Head trauma Viral illness Sinusitis Rx overuse Serious causes (increased ICP)
Causes of Increased ICP?
Masses, intrinsic increase of pressure
Increased ICP HAs attributes?
Worse HA w/ vomiting upon - lying down, 1st AM Wakens child at night Goes away standing up Worse w/ coughing/Valsalva/stooping Papilledema or Neuro deficits
What study checks for Intracranial bleeding?
Head CT
What study checks for posterior fossa lesions or subtle ABNLs concerning HAs?
MRI +- gadolinium contrast (SOC)
Basic HA TXT?
Lifestyle mods
NSAIDs/APAP
Migraine TXT?
1L - NSAIDS/APAP
2L - Triptans (Sumatriptan/Imitrex) Serotonin ag
Antiemetics
- Odansetron, Metoclopramide, Promethazine
CI to triptan therapy for migraines?
Focal neuro deficits w/ migraines
Basilar migraine signs > Syncope occurs = Stroke risk
Migraine prophylaxis considered if?
> 1 disabling HA/wk
Migraine prophylaxis Rx?
BB - propranolol (CI - asthmatic/DM) TCA Anticonvulsants (Valproic acid/topiramate) CCB AH - Cyproheptadine SSRI - Fluoxetine
Types of seizures?
- Generalized (begins diffusely)
- (Tonic/clonic - Grand mal OR Absence - Petit mal) - Febrile seizure (type of generalized)
- Partial (begins focally)
- (Simple - no altered consciousness)
- (Complex -Altered consciousness) - Status epilepticus (neuro emergency)
Simple partial seizure attributes?
Consciousness preserved
- Starts at specific anatomic focus +- spread
Simple partial seizure S/S?
Motor - Tonic/Clonic/myoclonic
Sensory, psychic, autonomic ABNLs
Complex partial seizure attributes?
Consciousness altered
- Starts at specific anatomic focus +- spread
Spread to whole brain = 2ndy generalized
Spread - Face to arm to leg = Jacksonian March
+- staring or automatisms
Types of generalized seizures?
Tonic-Clonic - Grand mal
Absence - Petit mal
Generalized - Tonic clonic seizure attributes?
Grand Mal seizure
- Tonic - Brief cry, Fall, LOC
- Clonic - Spastic, Rhythmic, symmetric shaking
- Postictal - Gradual return of consciousness
- EEG - NL when not seizing
Generalized Absence seizure - attributes?
Petit Mal
- Begins 4-6yo
- Short <15s blank stares and loss of awareness
- +- lip smacking/eye fluttering
- NO post-ictal state
- EEG - 3hz spike and wave activity
TXT Generalized Absence seizure w/?
Petit Mal
- Ethosuximide or Valproic Acid
What is a neurological emergency w/ ongoing seizures?
Status Epilepticus
Status epilepticus is?
Ongoing seizure activity or repetitive seizures w/out return of LOC for >30m
TXT of status epilepticus?
ABC, EKG, O2/SO2 monitoring, IV
Labs- CMP, CBC, GLU Anticonvulsant Rx, Toxicology
Rx
Rx TXT of status epilepticus?
Diazepam or Lorazepam - Rapid/short
Midazolam - IV/Rectal
Seizure eval req?
Febrile - r/o meningitis, encephalitis, brain abscess
Focal neuro S/S or Papilledema - CT before LP
CT - acute/blood in brain
MRI - subacute/brain lesions
Why CT before LP in a pt w/ seizures + focal neuro S/S or papilledema?
LP in a pt w/ ICP = herniation through foramen magnum
MRI is a study for what concerning neurology?
Brain lesions
What study is best for detecting brain bleeds?
CT
MC cause of seizures between 6mo-6yo?
Febrile seizures
Simple febrile seizure attributes?
Onset is generalized <15m occurring once/24h
- good prognosis - no relative complications
- No neuro Hx
Complex/atypical febrile seizure attributes?
Onset is focal >15m reoccurring w/in 24h
OR
Child has preexisting neuro Hx
Febrile seizure Mgmt?
Typically None - benign
+- rectal diazepam (prolonged or Hx of prolonged)
Sites where pathology for weakness/hypotonia occurs?
Anterior Horn cells
Peripheral nerves
NMJ
Muscle tissue
Conditions causing weakness/hypotonia at the Anterior Horn cells?
Spinal Muscular atrophy
Polio
Conditions causing weakness/hypotonia at the Peripheral Nerves?
Guillain-Barre syndrome
Charcot-Marie-tooth disease
Tick paralysis
Conditions causing weakness/hypotonia at the NMJ?
Myasthenia Gravis
Botulism
Conditions causing weakness/hypotonia at the muscular tissue?
Muscular dystophies
UMN destruction causes?
Loss of voluntary control - BUT not loss of movement
- Mild dysfx (slow/stiff motions)
- Severe dysfx (Stiff/ABNL postures/spastic)
Severe UMN destruction pt posture will be in what position?
Forearm flexed at elbow,
Wrist adduction to chest,
Leg extension and adduction
LMN destruction causes?
Absence of movement/Complete paralysis + hypotonia
LMN is common pathway for?
Muscle activity - Destruction = paralysis
Does LMN have abnormal posturing?
NO - paralyzed - progressive weakness/hypotonia
UMN is ass/w what pathway?
Corticospinal tract
LMN is ass/w what pathway?
Neuromuscular junctions
UMN lesions tone/DTR/Babinski?
Tone - Increased
DTR - Increased
Babinski - Present
LMN lesions tone/DTR/Babinski?
Tone - Decreased
DTR - Decreased
Babinski - Absent
Spinal muscular atrophy (SMA) pathology is at what site?
Anterior horn cells (degeneration)
Types of Spinal muscular atrophy?
Types
I - Wernig-Hoffman Dz
II - Kugelberg-Welander syndrome
III - Juvenile form
MC Spinal muscular atrophy type?
Type II - Kugelberg-Welander syndrome
Spinal muscular atrophy - Type I attributes?
Wernig-Hoffman Dz
- Severe infantile form
- die w/in 2y
Spinal muscular atrophy - Type II attributes?
Kugelberg-Welander syndrome
- Late infantile
- Slower than type I
- Survive to adulthood
Spinal muscular atrophy - Type III attributes?
Juvenile
- Chronic
- NL life
Spinal muscular atrophy is?
Progressive anterior horn cell degeneration
Clinical features of Spinal muscular atrophy?
Tongue fasciculations - Esp. sleeping
Proximal atrophy/weakness = progressive
Floppiness - decreased movement
Absent DTR
TXT of Spinal muscular atrophy?
None - PVT respiratory infection/support
Poliomyelitis pathology occurs at what site?
Anterior horn cells - destroyed in spinal cord
Poliomyelitis is due to?
A2M transmission of the poliovirus
Clinical features of Poliomyelitis?
Fever, Malaise, HA, URI s/s, N/V/D, myalgias
Meningeal signs
Prodrome > flaccid paralysis 3-8d after S/S
What is VAPP?
OPV - Vaccine associated paralytic poliomyelitis (No longer used)
USA - IPV used only
Guillain-Barre Syndrome pathology affects what site?
Peripheral nerves
Guillain-Barre Syndrome is?
Acute inflammatory demyelinating polyradiculoneuropathy.
MC cause of acute flaccid paralysis is PEDs?
Guillain-Barre Syndrome
Guillain-Barre Syndrome is due to?
Post infectious autoimmune peripheral neuropathy
respiratory/GI infection - Mycoplasma pneumonia or Campylobacter Jejuni
Guillain-Barre Syndrome clinical features?
Absent Reflexes
Symmetrical ASCENDING weakness (hrs-wks)
Hand/feet paresthesia
Bulbar/respiratory weakness - progressed
Mgmt of Guillain-Barre Syndrome?
Admit for observation +- respiratory support
IVIG, Plasma exchange, Immunosuppressive Rx
Resolution - 1yr
Charcot-Marie-Tooth Dz pathology is at what site?
Group of Peripheral nerve diseases
What pathway is affected 1st w/ Charcot-Marie-Tooth Dz?
Motor > sensation or autonomic
MC type of Charcot-Marie-Tooth Dz?
Type 1A
Clinical features of Charcot-Marie-Tooth Dz?
Ankle weakness/frequent tripping
PES CAVUS deformity (high arched feet)
Slow progression
Earliest nerves affected by Charcot-Marie-Tooth Dz?
Peroneal and tibial nerves
TXT of Charcot-Marie-Tooth Dz?
Support - foot bracing
PT
Tick paralysis is clinically similar to what neuro disease?
GBS
What does tick saliva do?
Blocks NM transmission causing acute motor neuron weakness
Clinical features of Tick paralysis?
Severe generalized flaccid weakness
- including ocular, papillary, bulbar paralysis
TXT of Tick paralysis?
Find/remove tick > motor fx returns
Myasthenia gravis is ass/w what pathologic site?
NMJ
Myasthenia gravis is?
Autoimmune - ABs block ACh Receptors at NMJ causing rapid muscle fatigue
Childhood types of Myasthenia gravis?
Juvenile Myasthenia gravis
Transient neonatal Myasthenia gravis
Congenital Myasthenia gravis
Transient neonatal Myasthenia gravis is due to?
Neonates born to mothers w/ Myasthenia gravis and have maternal anti-AChR
Congenital Myasthenia gravis is?
genetic mutations of NMJ (NO autoimmunity)
Myasthenia gravis presents as?
Ptosis, Diplopia, ophthalmoplegia
Extremity, Neck, face, jaw - weakness
Myasthenia gravis is dx how?
Tensilon test (Edrophonium) = ACh inhibitor - POS= temporary improvement in strength
TXT of Myasthenia gravis?
ACh inhibitors - Pyridostigmine
Thymectomy - Reduce AB burden
PO CCS or Immunosuppressive
Severe - plasma exchange/respiratory support
Botulism is due to?
Clostridium botulinum - neurotoxin
PVT of infantile botulism?
No honey/canned food <1yo
Botulism presents as?
1st signs - Constipation/poor suck/feed Hypotonia/weak Decreased Gag reflex, Eye movements, Pupil contract Ptosis Respiratory difficulty
Botulism pathology site?
NMJ
Types of muscular dystrophies?
Duchenne Muscular dystrophy
Becker Muscular dystrophy (milder than Duchenne)
What is Muscular dystrophy?
Lack of dystophin production - Genetic muscle diseases causing progressive myofiber degeneration and fibrotic tissue formation.
Duchenne Muscular dystrophy attributes?
X-linked
Dystrophin gene mutation
Asymptomatic in infancy
Delayed motor milestones
Duchenne Muscular dystrophy presents as?
2-3yo w/ awkward gait or cant run Gait is hyperlordotic/waddling Proximal leg weakness Clumsy, easily fatigued, weak muscles Pseudohypertrophy of calves Arm weakness by 6yo Wheelchair bound 12yo Ass/w Gower sign
Gower sign is ass/w what neuro condition?
Duchenne Muscular dystrophy
Why is Duchenne Muscular dystrophy ass/w Pseudohypertrohic calves?
Atrophy of thighs
Prader-willi syndrome is?
Severe neonatal hypotonia due to deletion of paternal Chromo 15q11q13
Prader-willi syndrome presents as?
Feeding problems > FTT
Small hands/feet
Boys - Small penis/testicles or cryptorchidism
Early childhood - obesity/hyperphagia
Weakness eval req?
Muscle enzymes
CSF
Muscle Bx
DNA blood test
What test is Dx for muscular dystrophy?
Muscle Bx
ABNL CSF results will point to what weakness d/o?
Polio or GBS
What muscle enzyme is elvated for muscular dystrophy?
CK elevated
MC symptom of concussion?
HA
Concussion is?
TBI resulting in rapid onset of short lived neuro impairment
Retrograde amnesia is?
Inability to recall events leading up to trauma
Anterograde amnesia is?
Cant form new memories after trauma
Second impact syndrome is?
Acute - sometimes fatal brain edema (increased ICP) when 2nd concussion occurs before 1st concussion heals fully.
RFs for concussion?
Artificial turf Football/Rugby Females playing - soccer/basketball BMI >27 Training <3hr/wk
RFs for recurrent concussion?
Hx of Concussion
Longer playing time
QB for football
1st 10d after a Concussion occured
Concussion SOC?
CT - Normal if a Concussion only (used to r/o)
Seizures ass/w Concussions are Benign or Pathologic?
If seizure occurs
- at time of injury - Good prognosis
- W/in 7d - Good prognosis
- After 7d - glial scarring > lead to epilepsy
When can a Concussion lead to epilepsy?
After 7d s/p Concussion pt has seizures = glial scarring
A Concussion symptoms usually last how long?
<72h
Most Concussion resolve how long?
7-10d
Protocol for returning back to sports?
Stepwise Q/24h
Day 1 - No activity - rest
Day 2 - Light aerobic exercise - no resistance pt
Day 3 - Sport specific exercise
Day 4 - Noncontact drills - resistance training
Day 5 - Full contact practice after medically cleared
Day 6 - Gameplay
Epidural Hemorrhage attributes
ONSET - mins-hrs
CT - Lens-shaped/football bleed compressing brain
TXT - Surgical evac/monitor
Epidural Hemorrhage S/S?
Lucid interval w/ progressive neurologic deficits
Subdural Hemorrhage attributes?
ONSET - Hrs
CT - Crescent shape bleed compressing brain
TXT - surgical evac
Subdual Hemorrhage S/S?
Focal neuro deficits
Intraparenchymal Hemorrhage attributes?
Bleeding inside the brain tissue
Intraparenchymal Hemorrhage S/S?
Depressed consciousness
Focal neurologic deficits
+- multiple other contusions
TXT of Intraparenchymal hemorrhage?
Supportive only
Subarachnoid Hemorrhage S/S?
Stiff neck
Worst HA of life
Late hydrocephalus
TXT of Subarachnoid Hemorrhage?
Supportive
What brain Hemorrhages have best prognosis?
Epidural
Subdural
4 types of neurocutaneous d/o’s?
Neurofibromatosis
Tuberous sclerosis
Sturge-Weber syndrome
Arnold-Chiari Malformation
Types of Neurofibromatosis?
Type 1 - (Von Recklinghausen disease)
Type 2 - (Rarer)
What is the genetic etiology of Neurofibromatosis Type 1?
Autosomal dominate mutation on chromo 17 on NF1 gene (neurofibromin) - BUT can occur w/ spontaneous mutation
NF1 gene codes for?
Neurofibromin
Neurofibromas are?
Schwann cells that develop into dome shaped papules
Cardinal features of Neurofibromatosis Type 1?
Café au lait spots
Axillary/inguinal freckles
Cutaneous neurofibromas
Lisch nodules (iris hamartomas)
Dx of Neurofibromatosis type 1 requires?
> 1 of
- Café au lait spots (>5 or >5mm size)
- Axillary/inguinal freckles
- 2+ Cutaneous neurofibromas
- 2+ Lisch nodules (iris hamartomas)
- Optic gliomas
- Family Hx of Neurofibromatosis
- Osseous lesions
What RAD is required of Neurofibromatosis?
Cranial imaging to r/o neoplasms
MC cause of death in pts w/ Neurofibromatosis?
Malignancy
Neurofibromatosis Type 2 genetic etiology?
AD mutation on Chromo 22 (NF2 gene- Merlin)
NF2 gene codes for what?
Merlin
Neurofibromatosis Type 2 is?
Intracranial/spinal tumors (schwannomas, meningimas, gliomas)
Neurofibromatosis type 2 presents as?
Posterior capsular or cortical cataracts
Skin lesions - plaque like, Sub-q nodules, cutaneous schwannomas
What is not found in Neurofibromatosis type 2?
Café au lait spots
Lisch nodules
axillary/inguinal freckling
Tuberous sclerosis genetic etiology?
AD - mutation of
- chromo 9 - TSC 1 gene - Hamartin
- chromo 21 - TSC 2 gene - Tuberin
Tuberous sclerosis is?
Hamartomas of brain, eyes, skin, kidneys, heart
What does TSC 1 gene code for?
Hamartin
What does TSC 2 gene code for?
Tuberin
Cardinal features of Tuberous sclerosis?
Facial angiofibromas (ADENOMA SEBACEUM)
MR
Epilepsy
- Common cause for infantile spasms
Tuberous sclerosis CNS manifestation?
Cerebral cortex TUBERS (dysplasia areas) = MR/epilepsy
Tuberous sclerosis SKIN manifestation?
Adenoma sebaceum (sml red nodules - nose/cheek)
Ash leaf spots - Hypomelanotic macules
Shagreen patch - Raised, rough plaques (low back/ass)
Tuberous sclerosis RENAL manifestation?
Renal angiomyolipomas - malignant transformation
MC cause of death of adults w/ Tuberous sclerosis?
Renal angiomyolipomas
Dx of Tuberous sclerosis?
XR, CT/MRI
Skin lesion Bx
TXT of Tuberous sclerosis?
Support
Seizure Meds
Sturge-Weber syndrome is?
ABNL leptomeningeal blood vessels(ANGIOMAS) overlay cerebral cortex
Is Sturge-Weber syndrome inherited?
NO
What is Sturge-Weber syndrome ass/w?
- -Nevus flammeus - ipsilateral port-wine stain on face over ophthalmic division of trigeminal nerve
- -Glaucoma
Sturge-Weber syndrome presents as?
Seizures - (MC)
- hemiparesis
- Stroke like episodes
- HA
- MR
- Learning disabilities
TXT of Sturge-Weber syndrome?
Nevus Flammeus = laser surgery
Seizures = anticonvulsants
Arnold-Chiari Malformation types?
Types
I - Cerenellar tonsils herniate through foramen magnum
II - Type 1 + Lumbar meningomyelocele
III - Type 1 + Occipital encephalocele
Arnold-Chiari Malformation may present as?
Early life - asymptomatic
Later life - Ataxia and vertigo, Hydrocephalus (obstruct)