Neuro 9 Flashcards
Describe acute intermittent porphyria
♣ Deficiency of Porphobilinogen (PBG) deaminase
• THINK: Acute intermittent = guys hollering “damn” (deam-inase) intermittently at A CUTE pretty big girl (PBG) walking by
♣ Symptoms – 5 P’s
• Painful abdomen, Port wine colored urine (due to increase PGB), Polyneuropathy, Psychological disturbances, Precipitated by drugs (CYP450 inducers), alcohol, and starvation
What do Anti-Hu antibodies suggest
Paraneoplastic syndrome
What sx usually precede Guillain-Barre?
Acute infection, GI or URI
What will you see in CSF of Guillain Barre
Elevated protein with few or no cells
Clinical presentation of Guillain Barre
♣ Ascending symmetrical paralysis over days to weeks
♣ Absent/depressed deep tendon reflexes
♣ Paresthesias
♣ Autonomic dysfuction (e.g tachycardia, urinary retention, and arrhythmias) occur in 70% of patients
♣ Respiratory compromise
♣ Neuropathic pain is common
Presentation of transverse myelitis
- Area of inflammatory demyelination in the spinal cord
- Motor and sensory loss below the level of the lesion with bowel and bladder dysfuntion
- Initially flaccid paralysis followed by spastic paralysis with hyperreflexia
- Patients may report a band of tingling or pain around the torso at the level of the lesion
Tx of Guillain Barre
Immunoglobulin or plasmaphoresis
Tx of Myasthenic crisis
Plasmapheresis or IVIg, plus corticosteroids
What cancer is associated with Lambert Eaton
Small cell lung carcinoma
If you see EBV DNA in CSF of AMS HIV patient, what should you think?
CNS lymphoma
What other childhood disorder is associated with absence seizures?
ADHD
CO poisoning affects what part of the brain?
Bilateral basal ganglia
What will you see on EMG in neuropathy
Rapidly firing fibers with decreased recruitment
there are less muscle fibers so remaining ones must fire more
What will you see on EMG in myopathy
Early recruitment
there are same amount of muscles but they are weaker
What is PRES
• Posterior reversible encephalopathy syndrome (PRES)
♣ Headache
♣ Altered consciousness (acute confusional state)
♣ Visual disturbances (cortical visual loss – blindness with preserved pupillary reactivity)
♣ Seizures
ALS causes damage to which part of the spinal cord
Anterior motor horn (LMN) and corticospinal tract (UMN)
Syringomyelia causes damage to what part of the spinal cord
- Anterior white commissure (loss of pain and temp)
- Anterior horn (LMN)
- Lateral horn of hypothalamospinal tract (Horners)
Damage to non-dominant parietal lobe causes what?
Hemispatial neglect
Damage to what causes Erb-Duchenne palsy
Superior trunk (C5-C6)
Muscles affected in carpal tunnel
LOAF
- Lumbricals 1-2
- Opponens pollicis brevis
- APB (abductor pollicis brevis)
- FPB (flexor pollicus brevis)
Brain vascular supply of upper extremities
MCA
Brain vascular supply of lower extremities
ACA
Foot drop is caused by damage to what?
Damage to common peroneal nerve (does dorsiflexion)
Describe what Cerebral Palsy is
o Is a static (non-progressive) central motor dysfunction
o Can be acquired or genetic
o Risk factors:
♣ Hypoxic ischemic insult to brain in perinatal period, prematurity, low birth weight, chorioamnionitis, prenatal viral infections, prenatal strokes
3 types of cerebral palsy
♣ Hemiparetic
• Weakness and spasticity seen on one side of the body
♣ Diparetic
• Spasticity in all four limbs, but affecting the legs much more than the arms
• Often of normal intelligence and less likely to have seizures
♣ Spastic Quadriplegic
• All four limbs are affected
• Seizures often occur in the first 48 hours of life
Clinical features of cerebral palsy
♣ Infants: flaccid weakness, asymmetric limb movements, or seizures
♣ Older children: spasticity, dystonic, developmental delay, drooling
What is considered normal IQ
100 with a standard deviation of 15
Triad of Tuberous sclerosis
♣ Triad: Angiofibromas, mental retardation, seizures
♣ Other symptoms: Ash-leaf spots, hamartomas of CNS and skin, angiomyolipoma
Neuro finding associated with Neurofibromatosis type 1
Optic nerve glioma
Neuro finding associated with Neurofibromatosis type 1
Acoustic schwanomma
Will you see miosis or mydriasis in 3rd nerve palsy?
Mydriasis
Parasympathetic (constrictors) travel with CNIII, so if you knock these out you get dilation
Pt has a seizure and you want to give prophylactic meds until you figure out what is going on, what do you give?
Phenytoin, Phosphenytoin, Valproic acid
Tx of venous sinus thrombosis
Anticoagulation
What are the 4 midline columns in the brainstem and presentation of damage to each
- Motor pathway (corticospinal tract) – Contralateral weakness
- Medial lemniscus – loss contralateral proprioception/vibration
- Medial longitudinal fasciculus – Ipsilateral intranuclear ophthalmoplegia
- Motor nucleus and nerve – Ipsilateral CN motor loss (3, 4, 6, 12 – midline motor divide into 12)
What are the 4 lateral (side) columns in the brainstem and presentation of damage to each
- Spinocerebellar pathway – Ipsilateral ataxia
- Spinothalamic – Contralateral pain/temp sensation loss
- Sensory nucleus of CN 5 – Ipsilateral pain/temp loss in face
- Sympathetic pathway – Ipsilateral Horner’s syndrome
Parts of the spinal cord affected + sx of ALS
Affects anterior horn (LMN) and corticospinal tract (UMN)
Blood supply of medial and lateral midbrain
PCA
Describe acute intermittent porphyria
♣ Deficiency of Porphobilinogen (PBG) deaminase
• THINK: Acute intermittent = guys hollering “damn” (deam-inase) intermittently at A CUTE pretty big girl (PBG) walking by
♣ Symptoms – 5 P’s
• Painful abdomen, Port wine colored urine (due to increase PGB), Polyneuropathy, Psychological disturbances, Precipitated by drugs (CYP450 inducers), alcohol, and starvation
Damage to PPRF causes what?
Eyes look away from side of the lesion
Pronator drift is a sign of damage to what?
Corticospinal tract (pyramidal tract)
Diagnose: sudden onset painless monocular vision loss with cherry red spot
Retinal artery occlusion
Describe path of corticospinal tract
♣ UMN from motor cortex descend to pyramid in the medulla
♣ Axons cross to contralateral side in the caudal medulla and descend via lateral cortico-spinal tract
♣ Enter the spinal cord at lateral white column and travel to the anterior grey horn
♣ Synapse to LMN (alpha motor neuron) which then exits via ventral spinal root to innervate muscle
Describe Tuberous sclerosis presentation
♣ Triad = Angiofibromas, mental retardation, seizures
♣ Other symptoms: Ash-leaf spots, hamartomas of CNS and skin, angiomyolipoma
Describe Sturge Weber syndrome
♣ Port-wine stain of the face in CN V1/V2 distribution
♣ Seizures/epilepsy
♣ Intellectual disability
♣ Glaucoma
Triad of Wernicke
Confusion, ataxia, ophthalmaplegia
Presentation of CJD
Rapid onset dementia + ataxia + startle myoclonus
Periodic sharp waves on EEG
What function is PPRF and Edinger Westphal nucleus involved in?
PPRF = voluntary eye movement EWN = parasympathetic pupillary reflex
What is one of the earliest and most unique sx of Parkinsons
Loss of smell
Location of damage / presentation of Friedrich ataxia
♣ Degeneration of cerebellum and spinal cord
• Ataxia, muscle weakness, loss of vibratory sense and proprioception, diabetes mellitus, hypertrophic cardiomyopathy, slurred speech, dysphagia
• Presents in childood with kyphoscoliosis
♣ THINK: Friedreich is fratastic (frataxin): he’s your favorite frat brother, always staggering and falling but has a sweet, big heart
Describe Meniere disease
♣ Increased pressure and volume of endolymph
♣ Features:
• Episodic vertigo with nausea and vomiting
• Ear fullness/pain
• Unilateral sensorineural hearing loss
• Tinnitus
Paraneoplastic cerebellar degeneration is usually associated with what cancers?
Gynecologic or small cell lung cancer
Tx of Guillain Barre
IV Ig or plasmapheresis
Tx of essential tremor
Beta blockers
Primidone
Self-medicated via alcohol
Apraxia is caused by damage where?
Cortex (posterior parietal)
