Med-Neuro Flashcards
Cerebral Salt Wasting etx
⬇︎Brain adrenergic output to Kidney –> ⬇︎PCT Na+ Reabsorption–> hypOvolemic hypONatremia
[Wernicke Korsakoff Syndrome] Clinical Presentation (3)
Wernicke problems come in a CAN of beer!
[Confusion & Confabulation]
Ataxia (Gait & Postural)
[Nystagmus + Oculomotor Dyf] (Opthalmoplegia)
beer = chronic alcoholism is most common cause
Causes of [Wernicke Korsakoff Syndrome] (2)
Wernicke Problems come in a CAN of beer!
[Thiamine B1 Deficiency] from (below) –> BL circuit dysfunction between mammillary bodies & ANT Thalamus:
- Chronic Alcoholism = MOST COMMON
- Giving [Glucose that doesn’t have B1] to a B1-deficient pt (i.e. homeless malnutrition pt)
Tx for [Wernicke Korsakoff Syndrome] (2)
- IV [Thiamine B1]
- Glucose administration
What’s the major complication of [SubArachnoid Hemorrhage] during recovery? How do you tx this?
Usually in the Suprasellar Cistern
Severe Cerebral Vasospasm 4-12 days post SAH onset –> morbidity vs. mortality. Prevent with [Nimodipine CCB]
Other complications: Rebleeding, SIADH, Seizures
Describe the Demographic for the HA:
Migraine-2
Tension
Cluster
Migraine = Female and Kids(will be bifrontal)
Tension = Female
Cluster = Male (100% O2 tx)
Describe the Onset for the HA:
Migraine
Cluster
Tension
Migraine = Variable but possibly during menstruation
Cluster = During Sleep (100% O2 tx)
Tension = When Stressed “think tense”
Describe the Location for the HA:
Migraine
Cluster
Tension
Migraine = POUND = [Pounding/One-3 Day Duration /Unilateral/Nausea/Disabling] + photo vs. phonophobia & [flashing dots aura]
Cluster = Behind 1 eye (100% O2 tx)
Tension = [Bilateral & Band-like around the head]
Describe the Character for the HA:
Migraine
Cluster (3)
Tension (2)
Migraine = POUND = [Pounding/One Day-3 day Duration/Unilateral/Nausea/Disabling] + photo vs. phonophobia & [flashing dots aura]
Cluster = [Excruciating, sharp & steady] (100% O2 tx)
Tension = Dull & tight
Describe the Duration for the HA:
Migraine
Cluster
Tension
Migraine = POUND = [Pounding/One-3 Day Duration /Unilateral/Nausea/Disabling] + photo vs. phonophobia & [flashing dots aura]
Cluster = 15 - 90 MINUTES (100% O2 tx)
Tension = 30 min to 7 DAYS!!!! (Tammy’s Entire Work Week)
Describe the Associated Sx for the HA:
Migraine
Cluster - 4
Tension
Migraine = POUND = [Pounding/One-3 Day Duration /Unilateral/Nausea/Disabling] + photo vs. phonophobia & [flashing dots aura]
Cluster = [Sweating/ Pupil Change / Lacrimation / Rhinorrhea]
Tension = [Muscle “Tension” in Head, Neck or Shoulders]
Which vessels are affected by [TUMTL-Transtentorial Uncal Medial Temporal lobe] Herniation? (3) What manifestations result from this?
[TUMTL Hernation–> Compression of [POP- PCA / Oculomotor CN3 / Paramedian Pontine vessels] –>
- [PCA compression] –> Occipital lobe infarct –> CTL homonymous hemianopsia w/Macular sparing
- Oculomotor CN3 compression –> [Ipsilateral “Down & Out” Eye + Ptosis + Dilated Pupil]
- Paramedian Pontine vessel compression –> Duret Hemorrhage
Which bone is associated with Epidural Hematoma?
Sphenoid
Violent Infant Shaking —> ________. This is characterized by what 3 things?
B: How is this differentiated from similar conditions?
Violent Infant Shaking –> [AHT- Abusive Head Trauma]! =
- Subdural Hemorrhage (from tearing bridging veins between Dura and Arachnoid)
- Retinal Hemorrhages Bilaterally (from congested retinal vein ruptures)
- POSTERIOR rib fractures
B: Usually Accidental Fall is not sufficient for Subdural Hemorrhage OR [BL Retinal Hemorrhage]
AHT is formely known as Shaken Baby Syndrome
What lab values differentiate seminomatous vs. NonSeminomatous Germ cell tumors?
seminomatous = ⬆︎bHCG
NonSeminomatous(yolk sac/choriocarcinoma/embryonal) = [⬆︎bHCG AND AFP]
[Thiamine B1] deficiency causes ____ and BeriBeri.
Describe BeriBeri (2)
[Wernicke Korsakoff Syndrome] and [BeriBeri]
BeriBeri (Wet vs. Dry vs. BOTH) is associated with…
- Heart involvement = WET
- Symmetrical Peripheral Neuropathy = DRY
[Thiamine B1] is needed to Decarboxylate a-ketoacids (carb metabolism)
Clinical Presentation for [Bells Palsy] (4)
Bells Palsy = Facial CN7 paralysis from inflammatory edema
Loss of F –> Entire half Unilateral Paralysis
Loss of A –> Hyperacusis
Loss of C–> DEC Eye lacrimation (tearing)
Loss of E –> Loss of ANT 2/3 Tongue Taste
FACE
- Facial Muscles
- Afferents(Somatic) from [External Auditory Canal (stapedius m.)] & [Ear Pinna (Pain/Temp)]
- Cry: Parasympathetics to [Lacrimal/Salivary/Sublingual/Submandibular/]
-
Eat: Taste from ANT 2/3 Tongue
* Note: Forehead sparing = Intracranial lesion and NOT Bells Palsy*
Early Findings of Alzheimer’s - 4
CLAV –> HANDU
Cognitive PROGRESSIVE ⬇︎
Language ⬇︎
Anterograde immediate memory loss
Visualspatial disorientation (loss in ur own neighborhood)
Onsets after 60 yo
Clinical Criteria for diagnosing Alzheimer’s -5
CLAV –> HANDU
- GOE 2 Cognitive deficits
- Worsening Memory
- Consciousness intact
- Onsets after 60 yo
- No other Systemic/Neuro DO to cause cognitive defects
Normal Pressure Hydrocephalus Sx (3); Which is earliest to present?
⬇︎CSF absorption –> Wacky, Wobbly & Wet!
Wacky (memory loss)
Wet (Urinary Incontinence from compressing periventricular cortico-cortical white fibers traveling to sacral micturition center)
Normal Pressure Hydrocephalus characteristics - 4
Wacky, Wobbly & Wet!
- Idiopathic
- Episodic
- Elderly
- Does not ⬆︎ SubArachnoid space volume
Etx: ⬇︎Arachnoid villi CSF Absorption vs obstruction
ANY Clinical Suspicion of Stroke warrants _____. Why?-2
NonContrast Head CT; Ischemic stroke benefits from Thrombolytics vs ICH requires neurosurgery
How do ICH (IntraCranial Hemorrhage) stroke appear on NonContrast Head CT? How long does this take?
[HYPERdense White]; IMMEDIATELY!
Ischemic Stroke = [hypOdense dark] and takes >24 hrs to appear
Clinical Presentation for Diabetic Ophthalmoplegia (3); Etx?
DM –> Oculomotor CN3 Central ISCHEMIA
- Ipsilateral Down & Out Eye
- Ptosis (from Levator Palpebrae paralysis)
- NORMAL PERRL (since Parasympathetic fibers are spared)
Ethosuximide Indication
Sux to have Silent Seizures
Silent (Absent) Seizures
Features of Absence Seizures -5
- Staring spells mid-activity
- Patient PAUSES activity
- Last < 20 seconds
- Not responsive to tactile/vocal stimulation
- NO recollection or postictal
- Provoked by Hyperventilation or photic stimulation / Dx = 3 Hz EEG spike*
- ADHD staring spells occur only DURING BOREDOM!*
Name a common trigger for Absence Seizures-2 ; Dx?
- Hyperventilation
- photic stimulation
; 3 Hz EEG spike
Why is it so important to recognize ____ in children with epilepsy?
ADHD; ADHD is common in Peds Epilepsy (includes Absence seizures) and if treated will ⬆︎ life quality
Newborn Galactosemia etx
inability of [(GALT) Galactose 1 Phosphate Uridyl Transferase] to convert Galactose1P –> Glucose, so Galactose accumulates
Susceptible to E.Coli !!!
S/S of newborn Galactosemia -7
- BL Cataracts
- Jaundice
- Vomiting
- Convulsions
- Failure to Thrive
- Hepatomegaly
- Irritability
Susceptible to E.Coli!!
[Cavernous Sinus Thrombosis] Presentation (3)
Infection of face vs teeth spreads thru facial veins –> cavernous sinus
- [CN3 / 4 / 6 / CN5B1 and B2] ipsilateral involvement
- Proptosis (protrusion of eye)
- HA
[Cavernous Sinus Thrombosis] etx
Infection of face vs teeth spreads thru facial veins –> cavernous sinus
Lacunar Stroke etx
lenticulostriate vessels perfuse [Be TIPC] (not Pons)
Lacunar Stroke= [Thrombotic HTN Arteriolosclerosis & Thrombotic microatheromas] of lenticulostriate vessels –> [cystic infarcts < 15 mm] –> Lacunar Syndrome
Describe the Lacunar Syndrome CP
lenticulostriate vessels perfuse [Be TIPC] (not Pons)
Lacunar Stroke= [Thrombotic HTN Arteriolosclerosis & Thrombotic microatheromas] of lenticulostriate vessels –> [cystic infarcts < 15 mm] –> Lacunar Syndrome
1A: Basal Ganglia–>Hemiballismus & involuntary writhing
1B: [Internal Capsule-POST limb/Pons/Corona Radiata]–> pure Motor stroke vs ataxia vs. clumsy hand-dysarthria
1C: ThalamuS VPL –> CTL Sensory Stroke
VPL=VentroPosteroLateral nc
What is Dejerine Roussy Syndrome
lenticulostriate vessels perfuse [Be I C]
S/p Lacunar Thalamus Sensory stroke eventually –> Severe Paroxysmal BURNING worst w/light touch = Allodynia
Clinical Presentation of Congenital Syphilis -7
- Frontal Bossing
- Deaf
- Saddle nose
- Rhinitis
- Hutchinson Mulberry Molars
- Liver/Spleen Dz
- Saber Shins
Clinical Presentation for Fetal Hydantoin Syndrome -6
Etx = Exposure to Phenytoin vs Carbamazepine during utero
- Microcephaly
- Cleft Lip / Palate
- Digital and Facial hypOplasia
- Hirsuitism
- Retardation
- Neonatal bleeding (Phenytoin ⬇︎ Neonate VitK)
Classic signs of Fetal Alcohol Syndrome - 4
- Microcephaly
- Small Palpebral fissures
- Long Smooth Philtrum
- Thin Upper Lip
Sturge Weber Syndrome Clinical Presentation -5
- Seizures
- Port Wine Stain vs Red Nevus along CN5 territory = congenital UL cavernous hemangioma
- CTL Homonoymous Hemianopsia
- Hemiparesis
- Ipsilateral Glaucoma
Tramline Gyriform Calcifications on CT
Sturge Weber Syndrome Dx
Tramline Gyriform Calcifications on CT
Sturge Weber Syndrome Tx -3
- Control Seizures
- ⬇︎ Intraocular pressure from Glaucoma
- Argon laser for [Port wine stain vs Red nevus along CN5 territory]
Tramline Gyriform Calcifications on CT
Tuberous Sclerosis Clinical Presentation (12)
HAMARTOMASSS
[Hamartomas benign]
[Angiofibroma on Face-triad] - image
Mitral Regurgitation
[Ash Leaf Macules]
[Rhabdomyoma Cardiac –> Valvular Obstruction]
Tuberous Sclerosis
AUTO DOM
Mental Retard-triad
[AngioMyoLipoma Kidney]
Seizures-triad
SEGA (SubEpendymal Giantcell Astrocytoma)
[Shagreen forehead patches]
Genetic Cause of Neurofibromatosis Type 1
[chromo 17 mutation]–> [NeurofibroMin loss]. NeurofibroMin tumor suppresses (RAS GTPase activating protein
Characteristics of Neurofibromatosis Type 1 (6)
CLAP ON type 1!”
- Neurofibroma PLEXIFORM
- Acoustic Schwannoma-Unilateral (HA/Tinnitus/Vertigo)
- [Optic n. Glioma]
- Lisch nodules
- [Cafe Au Lait Spots]
-
Pheochromocytoma
* Note: NF1 in Newborns will present w/Macrocephaly, ⬇︎Feeding ,learning idsabilities*
Neurofibromatosis Type 2
Clinical Presentation - 3
- [Bilateral Acoustic Schwannomas] (HA/Tinnitus/Vertigo)
- Multiple Meningiomas benign
- BL Cataracts
Bilateral Acoustic Schwannomas @ Cerebellopontine angle
In [Neurofibromatosis Type 1], Fleshy cutaneous neurofibromas are made of ______ which embryologically come from _____. These pts may also have hyperpigmented spots known as ____
In NF1, Fleshy cutaneous neurofibromas are made of Schwann cells, which are embryologically from Neural Crest. May also have [Cafe Au Lait Spots (image)]
Image: Cutaneous Neurofibromas & Cafe Au Lait Spots
Main features of Narcolepsy -4
- > [3x/week for more than 3 mo.] of sudden entry into REM sleep
- hypnagogic/hypnopompic hallucinations
- Sleep Paralysis (temporary paralysis after sleeping)
- Cataplexy
hypnogogic = when going to sleep
Cataplexy may be treated with ___-suppressing drugs. Name 2 examples
REM Sleep-Suppressing drugs; Antidepressants and Sodium Oxybate
List the 3 main causes of HemipLegia in Kids
- Seizure w/Todds Paralysis
- Hemorrhagic Stroke 2/2 AVM
- HemipLegic Migraine (Teens w/Fam hx, self-resolving)
Describe Todds Paralysis
Self-limited focal (ipsilateral UE and LE) paralysis after seizure that resolves naturally within 36 hours
What Dz occurs from [Tetrahydrobiopterin BH4] deficiency? Explain the etx
(PKU) Phenylketonuria; Dihydropteridine Reductase becomes deficient w/out [Tetrahydrobiopterin BHF] cofactor –> Inability to convert Phenylalanine –> Tyrosine
Phenylketonuria tx (2) ; Why is Newborn screening important for these?
- low phenylalanine diet
- BH4 supplementation
NEWBORN SCREENING–> early dx –> early tx –> Normal lives!!
PKU-Phenylketonuria S/S (4)
- Musty Odor
- Seizures (look for upward eye deviation)
- Eczema
- Retard
Newborn screening is ESSENTIAL for early dx of PKU
How do you diagnose PKU?
Tandem mass spectrometry of dried blood spots –> detects PKU products
Name the classic complaint pts with Presbycusis will give regarding conversations - 2
Can hear one-on-one BUT can not hear if there’s ANY background noise + BL tinnitus
Sensorineural hearing loss secondary to age
What conditions are associated with [Berry Saccular Aneurysm]? (5)
“Eating AppleBerries Can Sound Heavenly”
- ADPKD**
- [Ehlers Danlos Syndrome]
- HTN
- SAH (from Trauma > Berry Saccular Aneurysm)
- Coarctation of Aorta (associated w/HTN)
Image: Blood around Brainstem & Basal Cisterns
[Communicating Hydrocephalus] cause
[Meningitis vs. SAH/Intraventricular Hemorrhage] –>Disrupts communication of CSF with [Arachnoid Granulation Villi] ReAbsorption
[SubArachnoid Hemorrhage] Dx-3? Tx-2?
Usually in Suprasellar Cistern
Dx:
- NonContrast Head CT
- Lumbar Puncture revealing Xanthochromia (6 hrs after onset)
- Cerebral Angiography
Tx: [Endovascular Coiling/Stenting to stabilize aneurysm] + Nimodipine
Xanthochromia comes from Blood breakdown products
What’s the major complication of [SubArachnoid Hemorrhage] 24 hrs post onset?
REBLEEDING WITHIN 6 HRS –> MAJOR CAUSE OF DEATH!
Other complications: Rebleeding, SIADH, Seizures
What are Risk factors for PCiiH [Pseudotumor Cerebri Idiopathic Intracranial HTN] - 4
- Overweight Women taking OCP (will usually have Empty Sella Turcica)
- Tetracyclines
- Vitamin A OD (Isotretinoin)
- Growth Hormone
This HA will make you go Blind!
Lumbar puncture with CSF pressure of _____ = Intracranial HTN
> 250 mmH20
PCiiH [Pseudotumor Cerebri Idiopathic Intracranial HTN] Tx - 3
Big Girl with PCiiH just SAT on her problems
- Surgery (Shunt vs Optic N sheath fenestration)
- Acetazolamide (inhibits Choroid Plexus Carbonic Anhydrase)
- Topiramate (will also –> Wt loss :-) )
This HA will make you go Blind!
PCiiH [Pseudotumor Cerebri Idiopathic Intracranial HTN] Dx - 2
- Lumbar Puncture with opening pressure >250 mmH20 (from ⬇︎Arachnoid villi CSF absorption)
- MRI +/- MRV revealing BL tortuous Optic N
This HA will make you go Blind!
Papilledema is typically a ctx to Lumbar puncture. When is it not a ctx to Lumbar puncture? Explain
[PCiiH (Pseudotumor Cerebri Idiopathic Intracranial HTN)]; As long as there are no signs of obstructive/noncommunicating hydrocephalus or mass, then it is ok
LP with CSF opening pressure > 250 mmH20 = PCIIH
[Pseudotumor Cerebri Idiopathic Intracranial HTN] Clinical Presentation - 4
- [HA worst at sleep times (night/morning) & with head position ∆]
- Vision changes +/- Papilledema
- Pulsatile Tinnitus
- CN6 Abducens Palsy
This HA will make you go Blind!
[Syringomyelia central cord syndrome] etx ; CP-2?
Formation of [CSF filled syrinx cavity] in C8-T1 region of spinal cord –> damages [Ventral white commissure (crossing fibers for STT)] –> 1. [BL Cape distribution Pain/Temp Loss in Arms & Hands]
- ***Eventually Ventral Horns are also destroyed –> LMN signs (FAAW) - Fasciculations / Atrophy & Areflexia / Weakness
Parkinsonism Clinical signs (8)
PARK & hamp
[Pill Rolling Resting 4-6 Hz unilateral Tremor] worst with Rest & Mental Task
[Rigidity Cogwheel]
BradyKinesia
[AReflexia posturally] –>Shuffling Gait/Fall when turning or stopping
+
- hypOphonic speech
- Autonomic ⬇︎ (constipation / bladder problems / orthostatic hypOtension)
- micrographia
- poker masked face
- PARK = primary signs*
Name the Major UMN signs (5)
UMN signs = Weak MESH
Weakness
[Spastic Gait & Paralysis] (partially from disproportionate Extensor weakness)
[Exaggerated Reflexes (Babinski)]
Mental Status change
HemipLegia
Name the Lower Motor Neuron signs - 4
LMN signs (FAAW) - Fasciculations / Atrophy & Areflexia / Weakness
3 Main causes of Spinal Cord Compression
- DJD Disc Herniation (Smoking risk factor)
- [Epidural Staph a. Abscess (think IV drug user vs DM)]
- Tumor (Prostate/Renal/Lung/Breast/Multiple Myeloma mets)
Dx = MRI, Positive Straight Leg, Classic S/S
DJD=Degenerative Joint Disease
Causes of [Anterior Spinal Cord Syndrome] - 2
Thoracic AAA Repair vs Vertebra Burst Fracture
Describe the 3 main sx for [Brown Sequard Syndrome]
- Contralateral STT Loss of Pain/Temp 2 LEVELS BELOW ORIGINAL LESION
- Ipsilateral DCP Loss of 2TVP-2point/Touch/Vibration/[Position Proprioreception]
3. Ipsilateral CST Loss –> Muscle Weakness
Causes of [Brown Sequard Syndrome] - 3
- [(Extramedullary Tumor]
- Trauma
- [DJD Disc Hernation (Smoking risk factor)]
[Cauda Equina Syndrome] etx ; Clinical Presentation - 5
(Compression of S2 - S4 n. roots) –>
- Saddle Anesthesia (image)
- ⬇︎ Anocutaneous Reflex (perianal pinpoint does NOT cause anal sphincter contraction)
- Incontinence (urinary AND fecal)
- uL (uniLateral) Radiculopathy
- hypOreflexia (Conus Medullaris syndrome has HYPEReflexia)
Decompression required within 72 hours!!!
Where does Charcot Bouchard Aneurysms occur (4)
Charcot Bouchard Tears Pink
- Basal Ganglia
- Cerebellum
- Thalamus (shown in image below)
- Pons
Acute ICHH [Intraparenchymal CharcotBouchard HTN Hemorrhage] in image
What causes Hemiballismus
Damage (i.e. lacunar stroke) to Subthalamic nc., important in modulating basal ganglia output –> CTL Hemiballismus
Note: Basal Ganglia is in Subcortical nuclei
Etx of Huntington’s Disease
[AUTO DOM [Chromo 4 CAG repeats]] —> Degeneration of (Caudate nc. inside the ((I)ndirect Striatum w/ [Gross Caudate atrophy] ) –> [⬇︎ GABA release]
“Hunting 4 food is way too aggressive & dancey”
Huntington’s Dz Clinical Presentation (2)
- “Hunting 4 food is way too aggressive & dancey”*
1st: Aggressive Dementia w/ strange behavior
2nd: Dance-like Chorea mvmnts - AUTO DOM = Affects BOTH sexes equally!!*
When does Huntington’s Dz onset
30 - 50 y/o
AUTO DOM = Affects BOTH Sexes Equally!!
Describe Essential Tremor-2 ; What is it relieved by
- BUE Action Tremor worst w/Action (can also affect head & voice)
- No additional neuro ∆ present
relieved with EtOH
Onsets at 45 yo and 50% cases are AUTO DOM
Tx for Essential Tremor - 6
Propranolol > [PAT - Primodone vs Anticonvulsants vs Topiramate] > Benzo > Surgery
Onsets at 45 yo and 50% cases are AUTO DOM
Parkinson’s Dz Tx - 6
“Eat SALADS after you Park”
- [Levodopa (Dopamine Precursor) + Carbidopa]
- Amantidine
- Anticholinergics
- [Dopamine PostSynaptic Agonist] (NonErgot: Ropinirole vs. Pramipexole) & (Ergot:Bromocriptine)
- Selegiline
-
Surgery
- Pallidotomy: Destructive of [Globus Pallidus:internal]
- SubThalamic nuc. inhibition with electrode
- ANT Choroidal a ligation
Lesch Nyhan etx
MALE DO in which HGPRT deficiency –> ⬆︎ Purine –> Uric Acid accumulation
Presents in Males 6 mo. old with hypOtonia + vomiting eventually –> CROUG ( UE Self-Injury (Biting) / Choreoathetosis / Retardation / Gout / Obstructive Nephropathy
Lesch Nyhan Clinical Presentation - 7
Presents in Males 6 mo. old with hypOtonia + vomiting eventually –> CROUG ( UE Self-Injury (Biting) / Choreoathetosis / Retardation / Gout / Obstructive Nephropathy
Dx for Multiple Sclerosis - 5
- Clinical (SLUM SiiiN)
- T2 MRI: [Periventricular white matter demyelinating plaques with lipid laden macrophages]
- T1 MRI Black holes
- CSF Oligoclonal IgG bands
- Visual conduction velocity test
Sx will be disseminated in time and space
CP for [MIOS-MLF Internuclear Ophthalmoplegia Syndrome] (3)
[MIOS-MLF Internuclear Ophthalmoplegia Syndrome]
*[Impaired ADDuction of affected eye]
+
[Normal ADDuction of affected eye during [near reflex convergence]
+
*[Nystagmus of UNaffected eye when attempting to ABduct]
Image: L MIOS
Clinical Manifestation of Multiple Sclerosis (9)
Charcot classic triad of MS is a [SLUM SiiiN] !
Sensory sx (think BL Trigeminal Neuralgia)
Lhermittes sign = “electric tingling” down spine into arm & legs when chin is touched to chest
Uhthoff phenomenon (sx ⬆︎ during heat)
Motor sx
Scanning Speech
[Internuclear Ophthalmoplegia (MIOS)] / Intention Tremor / Incontinence
Neuritis Optic - (uL eye pain + vision loss + Marcus Gunn afferent pupillary defect) = ALSO RISK FACTOR
Which drugs are used to treat Multiple Sclerosis Exacerbation?-2 ;
Which are used for maintenance?-3
1st: High Dose IV Methylprednisolone
2nd: (Refractory): Plasmapharesis
Maintenance:
- β-interferon
- Glatiramer acetate
- Natalizumab
Myotonia Dystrophy Clinical Manifestation - 6
My Tonia, My Toupee, My TV Viewers, My Throat, My Ticker, My Testicles,
Tonia = MyoTonia = [⬇︎ relaxation after volitional muscle contraction with Weakness & Atrophy] (cant let go of doorknob)
Toupee = Frontal Balding
TV viewer = Cataracts
Throat = SEVERE DYSPHAGIA –> Aspiration PNA
Ticker = Arrhythmia
Testicle = Testicular Atrophy
Auto DOM CTG Repeat
Main features of Duchenne Muscular Dystrophy - 5
- CALF PSEUDOHYPERTROPHY requiring gower manuever
- Scoliosis**
- Cardiomyopathy
- Wheelchair by Teenage years
- Death by 20-30 yo 2/2 Cardiopulm failure**
- Onsets at 2 yo / X-Link Recessive deletion on Chromo Xp21*
- ** = also seen in Becker Muscular Dystrophy*
Main features of Becker Muscular Dystrophy - 2
- Scoliosis**
- Death by 40-50 yo 2/2 Cardiopulm failure**
- Onsets at 5 yo / X-Link Recessive deletion on Chromo Xp21*
- ** = also seen in Duchenne Muscular Dystrophy*
What are the Risk Factors for Alzheimer’s Dz - 6
CLAV–> HANDU
- > 60 yo
- Female
- Family hx
- Head Trauma
- Apolipoprotein E positive
- Down’s Syndrome (they have ⬆︎ [chromo 21 transmembrane amyloid precursor glycoprotein])
Frontotemporal Pick’s Dementia
Sx -2
Prounouced Frontal & Temporal lobe atrophy –>
[Socially inappropriate Behavior] + aphasia
OCCURS MORE IN FEMALES!!!
A: Demographic of Frontotemporal Pick’s Dementia?
B: Mode Of Inheritance
A: 50-60 yo Females (Alzheimer = > 60)
B: Auto Dominant
Dementia with Lewy Bodies (DLB) CP - 3
DLB at the DMV
- Dementia confusion periodically
- MichaelJFox Parkinsonism (PARK + hamp) tht does NOT respond to dopaminergic tx
- Visual Hallucinations
Lewy Body= [LABS (Lewy α-synuclein BodieS)] that are Eosinophilic intracytoplasmic accumulations
Tick Paralysis and Gullain Barre both present with ascending paralysis
What differentiates Tick Paralysis? - 3
Tick Paralysis has…
- NO Autonomic Dysfunction
- Normal CSF (GBS CSF=High Protein > 40)
- Can be Asymmetrical (GBS=Symmetrical)
CP of Cerebellar Damage - 7
Cere is def on GRINDRR
Gait Ataxia IPSILATERAL
Rapid alternating mvmnt impairment
Intention tremor/Dysmetria IPSILATERAL
Nystagmus IPSILATERAL (medial AND Lateral Vermis)
Dysarthria (Lateral Vermis only)
Rebound phenomenon (pt hits themself in face if flexing bicep and examiner releases arm-image)
Reflex Pendular (knee swings >4x after Deep tendon reflex is elicited)
Vermis is midline
Describe the “Clasp Knife” phenomenon ; What disease is this related to?
Rapid SPASTIC RESISTANCE to passive mvmnt of limb; UMN (Weak MESH) Pyramidal Tract dz
- Pyramidal Tract = Corticospinal and Corticobulbar*
- Pronator Drift also indicates Pyramidal Tract Dz*
Dx for Creutzfeldt Jakob disease - 6
- [PRNP prion protein] genetic testing
- EEG Biphasic vs Triphasic sharp wave complexes
- Postmortem brain biopsy
- ⬆︎CSF 14-3-3 proteins
- MRI Cortical Ribbons
- MRI basal ganglia hyperintensity
[Creutzfeldt Jakob Dz] etx
PrP (prion protein), normally in neurons as [a-helical structure] converts–> [INFECTIOUS Beta pleated sheets] –> Protease resistance –>
Vacuoles in [Gray Matter Neurons & Neutrophils] develop –> Cyst = [Spongiform Gray Matter]
[Creutzfeldt Jakob Dz] CP - 2
[RAPIDLY Progressive Dementia] + [STARTLE Myoclonus] –> DEATH
Can be Acquired vs. Inherited
[Amyotrophic Lateral Sclerosis] (Lou Gehrig’s) etx - 2
- Rare = [Superoxide Dismutase gene mutation] –> copper-zinc dysfunction —>[Upper AND Lower Motor Neuron Disease!]
- Common = Idiopathic
UMN Dz includes loss of neurons in motor nc. 5/9/10/12
DDx of Neuromuscular Weakness has 5 origins
Describe Upper Motor Neuron causes of Neuromuscular weakness - 4
DDx of Neuromuscular Weakness has 5 origins
Describe Anterior Horn Cell causes of Neuromuscular weakness - 4
DDx of Neuromuscular Weakness has 5 origins
Describe Peripheral Nerves causes of Neuromuscular weakness - 5
DDx of Neuromuscular Weakness has 5 origins
Describe Neuromuscular JUNCTION causes of Neuromuscular weakness - 4
DDx of Neuromuscular Weakness has 5 origins
Describe Muscle Fibers causes of Neuromuscular weakness - 5
Guillain Barre Tx - 2
IVIG vs Plasmapheresis
Guillain Barre CSF = HIGHLY ELEVATED Protein > 40
What Spinal Columns are affected in [Subacute Combined Degeneration]?-3 ; How does this manifest?-3
[SuBACute Combined Degeneration]
[Demyelinating lesions] in 3 Thoracic Spinal Columns:
- [Dorsal–> Loss of 2TVP]
- [Lateral CST –> UMN Weak MESH + Ataxia]
- Spinocerebellar
* FriEdreich Ataxia affects the SAME 3 columns*
Causes of [Subacute Combined Degeneration] (3)
[SuBACute Combined Degeneration]
1) B12 Deficiency
2) Copper Deficiency
3) AIDS/HIV
* Affects Dorsal / Lateral CST / Spinocerebellar Tracts (Combined)*
Postconcussive syndrome can occur __(length of time)__ after any TBI (Traumatic Brain Injury).
Describe CP for Postconcussive Syndrome - 4
hours-days;
- Continued Confusion/Amnesia
- HA
- Mood changes
- Vertigo
This is Self-Resolving
In pts with Traumatic Brain Injury (TBI), what’s the major cause of morbidity?
Diffuse axonal injury at Gray-White matter junction (since this is where density difference is highest)
USE MRI FOR DX
How long does it take Toradol to reach Max efficacy
3 hours
Dose = q4-6 hrs
You suspect a baby has ingested Botulinum spores
What’s the Clinical Presentation? - 4
- Descending Flaccid Paralysis (Floppy Baby)
- Ptosis
- Poor Suck & Gag Reflex w/drooling
- Constipation
Tx = IMMEDIATE Botulinum Ig
Spinal Muscular Atrophy etx and CP ; What’s the difference between Infant type and Adult type
[Spinal muscular atrophy] = specifically [ANT Horn Cell degeneration] from [Chromo 5 SMN1 and 2 gene mutations]–> LMN signs of FAW- Weakness/[atrophy & areflexia] /Fasciculations
*Infantile onset = (Werdnig Hoffman) –> [Auto Recessive FATAL condition –> Floppy Baby from defuse [Distal muscle atrophy]
*Milder childhood/adult onset types –> [Non-fatal Chronic Disability]
Why are Multiple Sclerosis pts at risk for BL Trigeminal Neuralgia
Demyelination may occur at Trigeminal nucleus –> BILATERAL neuralgia
Sx will be disseminated in space and time
After Getting Labs, NonContrast Head CT is next for dx unprovoked seizures
When would MRI be the better option? Name structural causes of epilepsy?-7
Use MRI in NONEmergent/elective situations;
- Temporal Sclerosis-shown in image
- Cortical Dysplasia
- TBI (Traumatic Brain Injury)
- Vascular Malformation
- Infection
- Tumor
- Infarction
[LEMS - Lambert Eaton Myasthenic Syndrome] etx
[Autoimmune attack against (Presynpatic Ca+ channel)–> No ACh release]
What other condition is [LEMS - Lambert Eaton Myasthenic Syndrome] associated with?
“LEMS has a good SOLC(soul)”
SOLC-Small Oat cell Lung Carcinoma
Name 4 Differentiating Factors for Myasthenia Gravis vs. [Lambert Eaton Myasthenic Syndrome]
- [LEMS] improves with exercise/exertion during the day!
- [LEMS] will show no imprvmnt with [Tensilon Edrophonium] injection OR ice pack
- [LEMS] nerve testing shows INC muscle responses
- [LEMS] has autonomic dysfunction (orthostasis, dry mouth, impotence)
What other condition is [Myasthenia Gravis] associated with?
May cause Thymoma (thymic hyperplasia)
[Myasthenia Gravis] etx ; Demographic?-2
Autoantibodies block and degrade [postsynpatic nicotinic ACh Receptors]] –> [⬇︎ motor end plate potential]
Presents in [Women 20-30] and [Men 60-80]
[Myasthenia Gravis] Clinical Presentation (6)
P DDD WF
[Ptosis
[Diplopia from Disconjugate gaze]
Dysarthria-bulbar dysfunction
Dysphagia w/nasal regurgitation-bulbar dysfunction
[Weakness Muscularly (Extraocular/RESPIRATORY/Proximal/ limbs /)]
[FATIGABLE Weakness Muscularly (worst w/repetition)]
Tx: Pyridostigmine AChesterase inhibitor
[LEMS - Lambert Eaton Myasthenic Syndrome] Clinical Presentation - 3
- Weakness of [Proximal limbs and trunk] mimicking myopathy, better with exercise
- Autonomic sx (Dry mouth /Orthostasis / Impotence)
- ⬇︎Deep Tendon Reflexes
You suspect a pt had a Stroke
After FIRST, ruling out Hemorrhagic stroke with _____, when should thrombolytic therapy be given? What should be given?
NonContrast Head CT; WITHIN 4.5 HOURS OF SX ONSET! ; IV Alteplase
How are HTN and DM mngmnt related to Acute CVA/TIA - 2
BP > 185/110 in setting of stroke can –> ICH - so Use Labetalol
&
Hyperglycemia augments brain injuries (so ONLY use NonDextrose IVF)
What is Therapeutic hypOthermia often used for? ; How low of temp can you go? ; SE of this?-4
Prevents hypoxic Brain injury in pts with [out of hospital cardiac arrest] ; 32C ;
- HYPERKalemia
- ⬇︎Cardiac Output
- ⬆︎Coagulation
- Immunosuppression
Homocystinuria Clinical presentation-5 ; tx-2?
auto recessive [Cystathionine synthase] deficiency –> Thromboembolism–> Stroke
- Marfanoid habitus (elongated limbs, arachnodactyly, scoliosis)
- Stroke
- Fair Hair & Eyes
- Ectopia Lentis
- Retarded
tx = [Pyridoxine B6] + AntiCoag
Homocystinuria dx-2
auto recessive [Cystathionine synthase] deficiency –> Thromboembolism–> Stroke
⬆︎ Homocysteine and Methionine
Name the Differences in CP between Marfan and Homocystinuria - 3
- Marfan are not retarded
- Marfan has no Stroke Risk
- Marfan are not fair complexion
Tay-Sachs etx ; CP-3
auto recessive B-hexosaminidase A deficiency –>
- Cherry Red Macula
- Seizures
- Retarded
Pronator Drift is a good indicator of what type of disease?
UMN Pyramidal Tract Dz (think stroke)
- Pyramidal Tract = Corticospinal and Corticobulbar*
- Clasp Knife phenomenon also indicates Pyramidal Tract Dz*
Etx of Parkinsons Disease
[LABS (Lewy α-synucleinBodieS)] accumulation in [substantia nigra pars compacta] –>degeneration –> ⬇︎Dopamine release and ⬇︎ stimulation of Striatum which –> allows Globus pallidus internal to continuously inhibit [VA/VL Thalamus from stimulating motor cortex]
There are 3 Dopamine D2 pathways in the brain
Name the pathways ; what overall effect do they have when activated?
Stimulation of….
Mesolimbic = Psychosis
Nigrostriatal = Mvmnt Coordination
Tuberoinfundibular = INHIBITS Prolactin when activated (if blocked –> infertility from hyperprolactinemia)
How is [Apolipoprotein E] related to Alzheimers
Apo E –> impaired synthesis and clearance of AB-amyloid —> INC risk for LATE onset Alzheimers
Alzheimer’s Dz etx (3)
Alzheimers etx = CHA
**Cleavage, Hemorrhage, (ACh⬇︎) **
- Cleavage of [chromo 21 transmembrane amyloid precursor glycoprotein] –> β-amyloid which accumulates–> [Neuritic Senile plaques] in temporal lobe early on.
- Hemorrhages Spontaneously occur in Occipital/Parietal lobes (image) from β-amyloid deposition in cerebral vessels
- ACh ⬇︎ in the [Basal nc. of Meynert] & Hippocampus 2/2 β-amyloid accumulation –> defective [Choline Acetyltransferase] in those areas –> Alzheimer Sx (CLAV–>HANDU)
What type of Hemorrhage is shown in image ; What is this typically associated with?
Lobar Hemorrhage (parietal) ; Amyloid Angiopathy 2/2 Alzheimers
Hypokalemic periodic paralysis CP-2
Occurs right after vigorous activity
- SUDDEN generalized muscle weakness +
- ⬇︎ Deep Tendon Reflexes
Occurs right after vigorous activity
Benzos can cause an uncommon SE known as Paradoxical Agitation. Describe this
[⬆︎Agitation, confusion and disinhibition] within a hour of benzo admin. GIVING MORE BENZOS WILL WORSEN THIS!
What is a Cephalohematoma? Tx?
Neonatal SubPeriosteal Hemorrhage limited to 1 cranial bone (i.e. does NOT cross suture lines) that onsets hours after birth and presents as scalp swelling +/- ⬆︎jaundice;
Tx = Nothing, since it self-resorbs within 2 weeks-3 mo.
Cerebellar infarction of medial vermis presents as _____-2
- Nystagmus
- Vertigo
Cerebellar infarction of Lateral vermis presents as _____-6
Cere is def on GRINDRR
Gait & Coordination Ataxia - IPSILATERAL
Rapid alternating mvmnt impairment
Intention tremor/Dysmetria - IPSILATERAL
Nystagmus (medial AND Lateral Vermis infarcts)
Dysarthria (Lateral Vermis only)
Rebound phenomenon
Reflex Pendular (knee swings >4x after Deep tendon reflex is elicited)
Intention tremor = worst as finger moves closer to target
Describe Features of BENA (Brocas Expressive NonFluent Aphasia) -4
- Right Hemiparesis
- Nonfluent speech
- Impaired Repetition
- Impaired Naming
BENA = Dominant Inferior Frontal
Describe Features of Wernickes Aphasia - 3
- R SUP homonymous quadrantanopia
- Comprehension problems
- Impaired Repetition
Conductive AND Wernicke Area = Dominant SUP Temporal
Describe Features of CONDUCTION Aphasia
VERY POOR Repetition
This is in addition to Fluent but many phonemic errors
Status Epilepticus clinical criteria?-2
- Single seizure > 5 min OR
- Cluster of Seizures w/ no return to baseline in between episodes
Image showing Cortical Laminar Necrosis s/p Status Epilepticus
What is the long term outcome of status epilepticus on the brain? ; Dx for this?
Cortical laminar necrosis ; MRI w/cortical hyperintensity
What is the most common cause of ICH in kids?
ArterioVenous Malformation
Identify ; Which are Lenticulate and which are Striatum?
“Gay People Cook!” = Basal Ganglia
Gay People=Lenticulate // People Cook= Striatum
- eg = Globus Pallidus
- es = Putamen
- d = Caudate
Tx for Cluster HA - 1st, 2nd and 3rd choice
1st = 100% O2 Nasal Canula
2nd = Sumatriptan
3rd = NSAIDs
Px = Verapamil
Px for Cluster HA
Verapamil
Also Px for Migraines
Describe CSF analysis for Guillain Barre
Glucose
Protein
WBC count
GPW
Normal Glucose / ⬆︎⬆︎Protein / Normal WBC
What are the Normal CSF lab ranges
Glucose
Protein
WBC count
GPW
Glucose: 40-70
Protein: <40
WBC: 0-5
Describe CSF analysis for Bacterial Meningitis
Glucose
Protein
WBC
GPW
⬇︎Glucose / ⬆︎Protein / ⬆︎WBC >1000
Describe CSF analysis for TB meningitis
Glucose
Protein
WBC count
GPW
⬇︎Glucose / ⬆︎Protein / ⬆︎WBC
Describe CSF analysis for Viral meningitis
Glucose
Protein
WBC count
GPW
Normal Glucose / ⬇︎ Protein / ⬆︎WBC (Lymphocytes)
Note: HSV actually has⬆︎Protein and ⬆︎ RBC also from temporal lobe destruction
Neonatal Intraventricular Hemorrhage occurs in premies less than ___ weeks gestation or less than ___ grams. Px?
< 30 weeks vs 1500g ; Antenatal Maternal Corticosteroids
- Normal Gestation = 40 weeks*
- Image: BL IVH & Dilated Vt*
Etx of Intraventricular Hemorrhage in premature babies less than _____ weeks or ___ grams
< 30 weeks vs 1500g ; Subependymal germinal matrix contains thin-walled vessels that easily rupture. Normally, these migrate before birth, but in premies they never have the chance which –> IVH –> ⬇︎Arachnoid CSF absorption –> Communicating Hydrocephalus
- Normal Gestation = 40 weeks*
- Image: BL IVH & Dilated Vt*
Choroid plexus cyst are identified ___ trimester and a marker for ____ in babies. How do they affect the baby?
second; Aneuploidy; They don’t affect baby. Regressess spontaneously and is benign
Dark holes = Cyst
What are the 7 major complications of Newborn Prematurity
Less than 32 weeks gestation specfically
BURPPIN
Bronchopulmonary Dysplasia
UcantBreathe (Neonatal Respiratory Distress Syndrome)
Retinopathy
- *P**atent Ductus Arteriosus
- *P**alsy CEREBRAL
Intraventricular Hemorrhage
Necrotizing Enterocolitis (⬆︎gastric residual volume with abd distension)
How do Traumatic Carotid Injuries occur?-3 ; Dx-2
Image: Carotid Dissection
- Penetrating Trauma
- Oropharyngeal trauma (falling w/object in mouth)
- Neck Strain (yoga, sports)
Dx = CT angio vs MR angio
These will present like Strokes
Lewy Body Dementia Tx
Rivastigmine AChinesterase inhibitor
What are the hallmark pathological findings for Alzheimers-2
[Tau Neurofibrillary tangles] & [Neuritic Senile Plaques]
Most serious complication of Guillain Barre? How do you determine when this complication gets really bad?
Respiratory Failure; FVC ≤ 20 mL/kg via SPIROMETRY means intubate!
HR, BP, Quadriparesis, FACIAL palsy are other serious complications
Levodopa is used to treat Parkinson’s Disease
Early SE?-3 ; Late SE
Early SE (HAD) = Hallucinations/Agitation/Dizziness
Late SE (5-10 yrs post tx) = Involuntary mvmnts
Dx for VitB12 deficiency - 3
- [⬆︎ Methylmalonic Acid levels]
- CBC showing Macrocytic Anemia
- Serum Vitamin levels
There are 3 Main causes of Spinal Cord Compression
Dx for Spinal Cord Compression-3
- MRI
- Classic S/S (BLE weakness, Worst w/spinal extension, better w/flexion, UMN signs)
- Positive Straight Leg
Note: In Acute Cord Compression, pts will have spinal SHOCK x3days = AReflexia and Flaccid paralysis
Brain Death is a clinical diagnosis and involves absent cortical and brain stem functions
T or F: Positive Deep Tendon Reflexes excludes a patient from being Brain Dead
FALSE! - Brain Death is limited to Cortex and Brainstem. Spinal Cord can still be functioning in Brain Death
[Myasthenia Gravis] Tx-4
P DDD WF
1st: Pyridostigmine AChesterase inhibitor
2nd: Cyclosporine
3rd: Thymectomy
**[Intubate + Plasmapharesis + IVIG + Steroids] if Crisis **
HemiNeglect Syndrome
Stroke in R Parietal Cortex (NonDominant hemisphere) –> Neglect of anything on the Left side
This is only in R handed people. It’s opposite for L handed
Usually Partial Seizures originate in a single hemisphere
Simple Partial Seizures may present as what 3 things?
- Motor ∆ (head turning) - no LOC
- Sensory ∆ (paresthesias)- no LOC
- Autonomic ∆ (sweating)- no LOC