1-CNS Flashcards
Describe Foster Kennedy Syndrome
Anosmia
IL Optic Atrophy
CL Papilledema
Lesion in anterior skull base or frontal lobe
What is a Marcus Gunn Pupil?
Afferent pupil defect (AFD)
- inturruption of optic pathway prior to chiasm (nerve or retinal disease)
- causes perception of dimming light and consequential pupil dilation
What is an Adie Pupil?
Tonic UL/BL pupil caused by lesion to the ciliary ganglion.
Poor constriction in response to light.
What disease is the Adie pupil associated with?
Holmes-Adie syndrome - benign (often familial seen in women)
- Adie pupil with depressed DTRs (often legs), anhidrosis, orthostatic hypotension, cardio autonomic instability
What is an argyll robertson pupil?
small, irregular pupils, only constrict for accommodation, but not in response to light
Caused by: neurosyphilis, lesion to CNIII descending fibers, EGW nucleus (MS)
What should be included in a differential for ptosis?
- Autonomics lesion: superior tarsal muscle(partial ptosis)
- CNIII Lesion: levator palpebrae
- NMJ: mysthenia gravis
- Muscular Dystrophy
Could also be a pseudoptosis: redundant skin folds associated with aging.
Describe the different manifestations of facial paralysis and their root causes?
Full hemi-facial paralysis: LMN symptoms (Bell’s Palsy), peripheral nerve lesion or brainstem below pons
Lower Facial Paralysis: UMN manifestation, forehead spared, lesion above pons
Describe the various primitive reflexes?
Grasp: stroking palm elicits grasp
Root: stroking cheek elicits nursing response
Snout: strike nose results in suckling response
Suck: touch lips elicits suck response
Palmomental: twitch of chin in response to stroking of palm
Glabellar sign (Myerson): tapping forehead elicits blinking
Which primitive reflex is always abnormal/pathologic when seen in adults?
grasp
What are the most worrisome abnormal physical findings in the elderly?
Grasp (alzheimers)
Babinski (UMN)
Tingling/Burning (positive findings are always bad)
Significant negative findings- e.g. weakness
Clumsiness
Describe some normal deficits found in the elderly
decreased vib sensation
depressed reflexes
some primitive reflexes
Describe presbycusis.
Is it a serious worry?
Elevated auditory threshold seen in most aging adults due to degeneration of hair cells in the organ of corti.
Not worrisome unless it greatly affects ADLs
Word for decreased sensation?
Hypoesthesia
Word for increased sensation?
Hyperesthesia
Word for unpleasant, abnormal sensation produced by normal stimulus?
Dysesthesia
Word for abnormal sensation (burning, prickling, crawling).
Paresthesia
Describe Guillain-Barre Syndrome
acute, idiopathic inflammatory polyneuropathy (primarily demyelinating motor polyneuropathy
ascending paralysis begins in feet. sometimes starts cranially
List some clinical features of Guillain-Barre syndrome
- Paresthesias (ill-defined back pain, tingling feet)
- Weakness
- Areflexia
- Facial diplegia
- Autonomic instability
What is uremic polyneuropathy?
“restless legs” - burning, cramping, itching crawling, creeping) –
commonly seen in people with renal failure due to build-up of MW toxins despite dialysis
Describe symmetric sensorimotor polyneuropathy.
Starts as “Stocking-Glove” neuropathy and then progresses from small fiber to large fiber (diabetic pseudotabes - loss of position sense)
What is amyotrophy?
Gradually progressive proximal leg weakness. Painful, no sensory loss
Describe some of the different possible toxic neuropathies.
Arsenic: sensorimotor peripheral polyneuropathy
Lead: wrist drop
Mercury: blindness, peripheral neuropathy
Thallium: alopecia, sensory and autonomic findings
Chemotherapeutics: vincristine, cisplatin, adriamycin.
What is the significance of Isoniazid?
anti-TB drug
B6 deficiency?
Describe Charcot Marie Tooth Disease
Inherited muscular atrophy
Type I: Demyelinating, stork leg deformity, foot drop, steppage gait, palpably enlarged nerves, peripheral polyneuropathy.
Type II: not as severe, axonal type, atrophy and polyneuropathy seen
What is porphyria?
Recurrent episodes of seizures, psych disturbances, abdominal pain, and motor neuropathy due to abnormal heme metabolism tthat is precipitated by barbituate drugs.
Inherited
What is the DDx for recurrent facial mononeuorpathies?
MS
Lyme Disease
Sarcoidosis
What is the DDx for bilateral facial mononeuropathies?
Giullain-Barre Myasthenia Gravis Baal meningitis (TB) Sarcoidosis Lyme Disease
Describe Tic Douloureux and its etiology?
Trigeminal Neuralgia
Sudden onset severe facial pain seen in middle age or later life.
Causes: idiopathic, multiple sclerosis, vascular, tumor
Often mistaken for sinus issues or tooth abscess
Define causalgia
trauma to nerve resulting in injury to sympathetic fibers
causes severe burning, dysesthetic pain, sudomotor, vasomotor, and atrophic changes
Describe the stages of Reflex Sympathetic Dystrophy.
I (Acute): increased temp, increased hair and nail growth, increased blood flow, rubor, edema, decreased ROM, may last up to 3 months
II(Dystrophic): hyperesthesia, cold intolerance, decreased temp, decreased hair growth, brittle nails, pale limbs, cyanotic, demineralization of bone,
III (Atrophic): decreased pain, hyperesthesia, smooth and glossy skin, muscle wasting, contractures.
What is the best course of treatment for Guillain Barre?
supportive care: PT, prevent DVTs (heparin, lovanox), respiratory therapy.
Plasmapharesis, IV IgG (to tone down immune response by flooding the body with antibodies), DO NOT USE STEROIDS
What is the best course of treatment for uremic polyneuropathy?
Treat symptoms
Renal Transplant
Best course of treatment for alcoholic polyneuropathy?
stop drinking
improve nutrition
What is the best course of treatment for Trigeminal neuralgia?
Meds: phenytoin, carbamazepine, clonazepam, baclofen, lamotrigine
Describe the proper course of treatment for reflex sympathetic dystrophy
- sympathetic block (surgical or chemical)
- regional IV meds
- TENs
- NSAIDs, propanolol, nifedipine,reserpine, guanethidine, prednisone, antidepressants, antiarrythmics, anticonvulsants, phenothiazines, gabapentin WTFFFFFFFFFF
Guillain Barre syndrome pathology?
Lymphocytic infiltration
Segmental demyelination
Axonal Degeneration
List the major motor (muscle disorder) peripheral neuropathies to note?
Guillain Barre
Motor Neuron Disease
Porphyria
Tick Paralysis
List the two major Cranial caused peripheral neuropathies
Guillain Barre
Diptheria
Describe Spinal Muscular Atrophy.
Degeneration of anterior horn cells as a result of a deletion gene
What are the two common types of spinal muscular atrophy?
Werdinger-Hoffman
Kugelberg-Welander
Describe Werdnig-Hoffman disease?
Floppy baby, evident in 1st weeks of life, death within a few years.
Progressive weakness, weak cry, dysphagia, respiratory failure
Describe Kugelberg-Welander
onset later in childhood
Describe the proper ways to dx a spinal muscular atrophy disorder.
H&P, muscle biopsy, EMG
Describe ALS (amyotrophic lateral sclerosis)
Degeneration of CST and anterior horn cells.
Describe Syringomelia
chronic progressive tubular cavitation of spinal cord.
Shawl-like pain and temperature loss.
What ailment is commonly seeen along with syringomelia?
Arnold-Chiari Malformation - congenital anomaly,
downward elongation of hindbrain into cervical column
Also seen with spina bifida and hydrocephalus
List some common causes of Subacute combined degeneration
B12 deficiency, pernicious anemia, myelin loss in the posterior columns
Describe Friedriech’s Ataxia
Hereditary disease resulting in deficiency in frataxin.
Degeneration of posterior funiculi, lateral CST, Spinocerebellar tracts, dorsal roots, and clarkes column.
Describe the clinical features of Friedreichs ataxia.
Areflexia Ataxia Loss of position sense Nystagmus Scoliosis Pes Cavus Cardiomyopathy
Describe conus medullaris syndrome
often occurs with cauda equina syndrome
- paralysis in the saddle region,
Describe ASA syndrome
Anterior Spinal Artery Syndrome
Cause by artery disease
Resuting in paralysis and dissociative sensory loss
What is the most common CNS tumor in the age group of infancy to adolescence? (0-20)?
Supratentorial: Glioma of cerebral hemisphere (10-14%)
Infratentorial: Astrocytoma (15-20%)
Medulloblastoma (14-18%)
What is the most common CNS tumor in the middle aged population (20-60)?
Supratentorial: Glioblastoma (25%), Meningioma (14%), Astrocytoma (13%)
Infratentorial: Metastases (10% – higher than that),
What is the most common CNS tumor in the old aged population (>60)?
Supratentorial: Glioblastoma (35%)
Meningioma (20%)
Metastases (10% – higher though)
Infratentorial: Acoustic neuroma (20%)
What is the etiology of the majority of Extradural spinal cord tumors?
Usually a metastatic carcinoma (breast, prostate, lung, GI), or lymphoma, myeloma, neurofibroma.
What is the etiology of extramedullary spinal cord tumors?
Neurofibromas and Meningiomas
What is the etiology of the majority of intramedullary spinal cord tumors?
Ependymomas and Gliomas
What is the #1 Primary Brain Tumor (meaning it originates in the brain)?
Gliomas (~60%) (incl.—glioblastoma, astrocytomas, ependymomas, medulloblastomas, oligodendrocytomas)
Note: adamant that metastatic carcinomas are far underrepresented due to the research facilities only getting the complex cases
Discuss the features of Astrocytomas covered in lecture.
Type of glioma that comprises of nearly 60% of all primary intracranial tumors.
Low and High Grade
- High = glioblastoma multiforme (GBM)
Prognosis is best predicted by necrosis and rate of growth