Internal Med- Neurology Flashcards

1
Q

How would you define stroke?

What is the etiology?

A

Stroke is the sudden onset of a neurological deficit from the death of brain tissue.
Stroke is caused by a sudden blockage in the flow of blood to the brain in 85% of cases and by bleeding in 15% of cases.

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2
Q

Where can the Emboli that cause a stroke originate from?

A
  • Heart: atrial fibrillation, valvular heart disease, or a DVT paradoxically getting into the brain through a patent foramen ovale (PFO).
  • Carotid stenosis
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3
Q

Which blood vessel is usually affected by a stroke?

A

Middle cerebral artery (MCA) stroke (more than 90% of cases):

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4
Q

What Sx does a Middle cerebral artery (MCA) stroke elicit?

A

-Weakness or sensory loss on the opposite (contralateral) side of the lesions causing stroke.
-Homonymous hemianopsia: loss of visual field on the opposite side of the stroke. A left-sided MCA stroke results in loss of the right visual fields. The eyes can’t see the right side, so the eyes deviate to the left. Hence the eyes “look towards the side of the lesion.”
-Aphasia if the stroke occurs on the same side as the speech center. This is the left side in 90% of patients.
Speech is controlled by the same side as “handedness.” Right-handed people (left brain dominant) have a speech center on the left-hand side of the brain.

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5
Q

What Sx does an Anterior cerebral artery (ACA) stroke elicit?

A
  • Personality/cognitive defects such as confusion
  • Urinary incontinence
  • Leg more than arm weakness
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6
Q

What Sx does a Posterior cerebral artery (PCA) stroke elicit?

A

Ipsilateral sensory loss of the face, 9th and 10th cranial nerves
Contralateral sensory loss of the limbs
Limb ataxia

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7
Q

What is the best initial test when suspecting a stroke?

What is the most accurate test?

A

The best initial test in any kind of stroke is a CT scan of the head without contrast. The most accurate test is an MRI. CT scan is done first, not because it is the most sensitive test for stroke, but in order to exclude hemorrhage as a cause of the stroke prior to initiating treatment.

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8
Q

What is the latest time that thrombolytics can be initiated in a Pt with a Stroke?

A

4,5 hours after the onset of stroke.

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9
Q

How would you Mgx nonhemorrhagic (Ischemic) and hemorrhagic stroke?

A
  • Less than 3 hours since onset of stroke: thrombolytics (TPA)
  • More than 3 hours since onset of stroke: aspirin
  • Hemorrhagic stroke: nothing. Hemorrhagic stroke has no treatment to reverse it. Surgical drainage will not help outside posterior fossa.
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10
Q

What is your next best step if a Pt with a stroke is already on Aspirin?

A

Add dipyridamole
or
Switch to clopidogrel

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11
Q

If Pt’s Sx (stroke Sx) last <24hr and then resolve, what is the next best step in Mgx?

A

Its a TIA so Tx with;
Aspirin + dipyridamole
or
Switch to clopidogrel if Pt is already on Aspirin

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12
Q

If a Pt you suspect has a stroke comes in after 4.5 hours, what is the next best step in Mgx?

A

Remove clot via catheter. Catheter retrieval pulls the clot out like a corkscrew. It is useful up to 6–8 hours after stroke, but angioplasty is not. Angioplasty would rupture the vessel.

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13
Q

Besides blood thinners (aspirin or clopidogrel), which medication should be given to every patient with a stroke?

A

A Statin.
Although target-based therapy for lipid management is unclear at this time, we want to bring the LDL to at least under 70.

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14
Q

How does acute blood appear on a CT? If there is a need for contrast, what type of contrast is needed?

A

Acute blood appears white on a CT scan. Contrast is not needed to detect blood.

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15
Q

What tests can be done to evaluate causes of a Stroke?

A
  • Echocardiogram
  • EKG
  • Holter monitor (24 to 48 hour ambulatory EKG)- If the initial EKG is normal, a Holter monitor should be performed to detect atrial arrhythmias with greater sensitivity.
  • Carotid duplex ultrasound
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16
Q

In a Stroke Pt, if the Echocardiogram reveals a thrombi, what is the next best step in Mgx?

A

Heparin followed by Warfarin to an INR of 2 to 3. Rivaroxaban and Dabigatran are alternative medications.

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17
Q

In a Stroke Pt, if the EKG reveals Atrial fibrillation or flutter, what is the next best step in Mgx?

A

Tx with a NOAC or warfarin as long as the arrhythmia persists.

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18
Q

In a Stroke Pt, if the EKG is normal, what is the next best step?

A

a Holter monitor should be performed, as it detects atrial arrhythmias with greater sensitivity.

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19
Q

In a Stroke Pt, if the Carotid duplex ultrasound reveals stenosis more than 70%, what is the next best step in Mgx?

A

Endarterectomy.
If the stenosis is 100%, however, no intervention is needed. There is no point in opening a passage that is 100% occluded.

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20
Q

In a Stroke Pt, if the Carotid duplex ultrasound reveals stenosis less than 50%, what is the next best step in Mgx?

A

No intervention is needed.

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21
Q

Which of the following has greater value in the Mgx of Stroke, Endarterectomy or Angioplasty and Stenting?

A

Endarterectomy is superior to carotid angioplasty and stenting.

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22
Q

What are some of the clinical features that would make a Migraine be the cause of a Pt’s headache?

A

Visual disturbance (flashes, sparks, stars, luminous hallucinations), photophobia, aura, relationship to menses, association with food (chocolate, red wine, cheese). May be precipitated by emotions

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23
Q

What are some of the clinical features that would make a Tension headache be the cause of a Pt’s headache?

A

A headache with no physical findings.

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24
Q

What are some of the clinical features that would make a Cluster headache be the cause of a Pt’s headache?

A

Frequent, short duration, high intensity headaches, with men affected 10 times more than women. Tearing eye with rhinorrhea; Horner syndrome occasionally

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25
Q

What are some of the clinical features that would make Giant cell (temporal) arteritis be the cause of a Pt’s headache?

A

Visual disturbance, systemic symptoms such as muscle pain, fatigue, and weakness. Jaw claudication. Tenderness of the temporal area

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26
Q

What are some of the clinical features that would make Pseudotumor cerebri be the cause of a Pt’s headache?

A

Obesity, venous sinus thrombosis, oral contraceptives, and vitamin A toxicity. Mimics a brain tumor with nausea, vomiting, and visual disturbance.

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27
Q

How would you Dx Pseudotumor cerebri?

A

CT or MRI without an intracranial mass lesion and a lumbar puncture (LP) showing increased pressure. Only the pressure is abnormal. The CSF itself is normal.

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28
Q

What is the most accurate Dx test for Giant cell arteritis and how would you Mgx it?

A

It is associated with a markedly elevated ESR and the most accurate test is a biopsy.
It is critical to start steroids (prednisone) without waiting for biopsy in giant cell arteritis.

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29
Q

How would you Mgx a Tension headache?

A

NSAIDs and other analgesics

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30
Q

How would you Mgx a Migraine?

A

Triptans (sumaptriptan, eletriptan, almotriptan, zolmitriptan) or ergotamine as abortive therapy

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31
Q

How would you Mgx a Cluster headache?

A

Triptans, ergotamine, or 100% oxygen as abortive therapy

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32
Q

How would you Mgx a headache from Pseudotumor cerebri?

A

weight loss; acetazolamide to decrease production of cerebrospinal fluid. Steroids help. Repeated lumbar puncture rapidly lowers intracranial pressure. Place a ventriculoperitoneal shunt or fenestrate (cut into) the optic nerve if medical therapy does not control it.

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33
Q

What would you give to provide prophylaxis for Cluster headaches?

A

Verapamil

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34
Q

What is the indication for prophylactic Tx for Migraines?

What is the best preventive therapy?

A

More than 3 migraine headaches per month.

Propranolol.
Other preventive medications are:
-Calcium channel blockers
-Tricyclic antidepressants (amitriptyline)
-SSRIs, topiramate
-Botulinum toxin injections
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35
Q

What is Trigeminal Neuralgia and what are its clinical features?

A

Trigeminal neuralgia is an idiopathic disorder of the fifth cranial nerve resulting in severe, overwhelming pain in the face. Attacks of pain can be precipitated by chewing, touching the face, or pronouncing certain words in which the tongue strikes the back of the front teeth. Patients describe the pain as feeling as if a knife is being stuck into the face.

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36
Q

How would you Tx Trigeminal Neuralgia?

A

Oxcarbazepine or Carbamazepine

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37
Q

What is Postherpetic Neuralgia and how is it Mgx?

A

It’s a pain syndrome from Herpes zoster reactivation (Shingles),
after resolution of the vesicular lesions in about 15% of cases.

Tx-
To reduces incidence; acyclovir, famciclovir, or
valganciclovir, *not steroids
For pain; tricyclic antidepressants, gabapentin, pregabalin, carbamazepine, or phenytoin

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38
Q

When is the Zoster vaccine indicated and how is it different from the Varicella vaccine?

A

Zoster vaccine is indicated in all persons above the age of 60 to prevent herpes zoster (shingles). This vaccine is similar to the varicella vaccine routinely administered to children to prevent chicken pox or varicella, except that the dose is much higher.

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39
Q

In most cases, why is an EEG not the right answer for a Pt with Seizures?

A

There is no point in doing an EEG to identify the cause of a seizure if there is a clear metabolic, toxic, or anatomic defect causing the seizure. In other words, what would be the point of doing an EEG if the patient had hyponatremia or a brain lesion? You have already found the cause of the seizure.

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40
Q

What is the difference between Seizures and Epilepsy?

A

Seizures of unclear etiology are called epilepsy. If there is a clear cause, it is not epilepsy.

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41
Q

What is the initial therapy for a persistent seizure (Status Epilepticus)?

A

A benzodiazepine such as lorazepam or diazepam intravenously.

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42
Q

If a persistent seizure (Status Epilepticus) persists after giving a benzodiazepine, what is the next best step?

A

Give phenytoin or fosphenytoin

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43
Q

What is the difference between phenytoin and fosphenytoin?

A

Fosphenytoin and phenytoin have the same efficacy, but fosphenytoin has fewer adverse effects compared to phenytoin. Like lidocaine, phenytoin is a class 1b antiarrhythmic medication. When given intravenously, it is associated with hypotension and AV block. Fosphenytoin does not have these adverse effects and can therefore be given more rapidly.

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44
Q

If benzodiazepines and fosphenytoin do not stop a seizure, what is the next best step?

A

Administer phenobarbital

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45
Q

If benzodiazepines, fosphenytoin and phenobarbital do not stop a seizure, what is the next best step?

A

Use a neuromuscular blocking agent such as succinylcholine, vecuronium, or pancuronium to allow you to intubate the patient and then give general anesthesia such as midazolam or propofol. The patient must be placed on a ventilator before the administration of propofol, which can stop breathing.

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46
Q

What are the 4 different levels of therapy (in order) for persistent seizures (Status Epilepticus)?

A
  1. Benzodiazepine
  2. Fosphenytoin
  3. Phenobarbital
  4. General anesthesia (Preceded by a neuromuscular blocking agent such as succinylcholine to allow you to intubate the patient)
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47
Q

How can seizures be classified?

A
  1. Generalized seizures (grand-mal)- tonic-clonic seizures have varying phases of muscular rigidity (tonic) followed by jerking of the muscles of the body for several minutes (clonic).
  2. Partial seizure- Like the name implies, this is a seizure that is focal to one part of the body. For instance, a patient may have a seizure that is limited just to an arm or leg. Partial seizures can either be simple (intact consciousness) or complex (loss or alteration of consciousness).
  3. Absence (petit-mal) seizure: Consciousness is impaired only briefly. The patient often remains upright and gives a normal appearance or seems to be staring into space. Absence seizures occur more often in children.
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48
Q

How would you Mgx epilepsy?

A

levetiracetam, phenytoin, valproic acid, and carbamazepine all have nearly equal efficacy. Levetiracetam has the fewest adverse effects.

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49
Q

It is not necessary to begin antiepileptic drugs for a single seizure. What are some of the exceptions in which you should start after a single seizure?

A
  • Presentation in status epilepticus or with focal neurological signs
  • Abnormal EEG or lesion on CT
  • Family history of seizures
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50
Q

Which drug is best for absence seizures?

A

Ethosuximide

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51
Q

Can we Tx Alcohol withdrawal seizures with long-term antiepileptic
drugs? If so, which drug?

A

Alcohol withdrawal seizures are not treated with long-term antiepileptic drugs.

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52
Q

In what situation can a Pt discontinue seizure medication?

What is the best way to tell if there will be reoccurrence?

A

The standard of care is to wait until the patient has been seizure-free for 2 years.
A sleep deprivation EEG is the best way to tell if there is the possibility of recurrence. Sleep deprivation can elicit abnormal activity on an EEG, but the test lacks high sensitivity.

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53
Q

A 38-year-old man is evaluated for seizures. He achieves partial control with the addition of a second antiepileptic medication. He drives to work each day.
What do you do about his ability to drive?

a. Confiscate his license.
b. Allow him to drive if he is seizure-free for 1 year.
c. Allow him to drive as long as his seizure history is noted on his license.
d. Recommend that he find an alternate means of transportation.
e. Do not let him leave the office unless he is picked up by someone; no further driving.
f. Allow him to drive as long as he is accompanied.

A

*D.
You do not have the right, as a physician, to confiscate a patient’s driver’s license. The rules on seizure disorder and motor vehicles vary from state to state. Reporting his condition to the department of motor vehicles does not have the same clarity as, for instance, reporting child abuse, in which the doctor is legally protected for all reports made in good faith. You cannot hold a patient (incarcerate) for seizures in the way that you can for tuberculosis. Being accompanied in a car does not prevent seizures.

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54
Q

Give a briefing of Subarachnoid hemorrhage (SAH)?

A

It is caused by the rupture of an aneurysm that is usually located in the anterior portion of the circle of Willis. Aneurysms are present in 2% of routine autopsies. The vast majority never rupture. They are more frequent in those with:
-Polycystic kidney disease
-Tobacco smoking
-Hypertension
-Hyperlipidemia
-High alcohol consumption
What provokes a rupture is not clear in the majority of cases.

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55
Q

What clinical features would make Subarachnoid hemorrhage (SAH) the most likely Dx?

A

Sudden onset of an extremely severe headache with meningeal irritation (stiff neck, photophobia) and fever.

  • Fever is secondary to blood irritating the meninges.
    meninges. Loss of consciousness occurs in 50% from the sudden increase in intracranial pressure. Focal neurological complications occur in as many as 30%.
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56
Q

When suspecting Subarachnoid hemorrhage (SAH), what is best initial test?
What is the most accurate test?

A

Best initial test: CT without contrast (95% sensitive).
Most accurate test: lumbar puncture showing blood. *LP is necessary only for the 5% that have a falsely negative CT scan.

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57
Q

What are some of the findings you would expect to find in the CSF of a Pt with Subarachnoid hemorrhage (SAH)?
How would you ddx some of the changes with those of meningitis?

A

Xanthochromia is a yellow discoloration of CSF from the breakdown of red blood cells (RBCs) in the CSF.
The CSF in SAH will have an increased number of WBCs, which can mimic meningitis. However, the ratio of WBCs to RBCs will be normal in SAH. Normal ratio: **One WBC for every 500 to 1,000 RBCs.
When the WBC count exceeds the normal ratio, you should suspect meningitis.

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58
Q

Why is a contrast not necessary when looking for blood on a CT or MRI?

A

Contrast on CT or MRI improves detection of mass lesions such as cancer or abscess but not blood.

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59
Q

What ECG finding is common with Intracranial Bleeding?

A

Large or inverted T waves suggestive of myocardial ischemia (cerebral T waves). This is thought to be from excessive sympathetic activity.

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60
Q

In case of Intracranial Bleeding, how do you precisely tell which vessel ruptured?

A

CT angiography, standard angiography with a catheter, or MRA.

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61
Q

How would you Mgx Intracranial Bleeding?

A
  1. Nimodipine (calcium channel blocker) prevents subsequent ischemic stroke.
  2. Embolization (coiling) uses a catheter to “clog up” the site of bleeding to prevent a repeated hemorrhage. An interventional neuroradiologist places platinum wire into the site of hemorrhage. Embolization is superior to surgical clipping in terms of survival and complications.
  3. Ventriculoperitoneal shunt: SAH is associated with hydrocephalus. Place a shunt only if hydrocephalus develops.
  4. Seizure prophylaxis: Phenytoin is generally given to prevent seizures. If the question asks “Which of the following is indicated?” antiepileptic
    therapy is the answer, although controversial.
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62
Q

A woman comes to the emergency department with a severe headache starting one day prior to admission. On physical examination she has a temperature of 103°F, nuchal rigidity, and photophobia. Her head CT is normal. LP shows CSF with 1250 white blood cells and 50,000 red blood cells.

What is the most appropriate next step in the management of this patient?

a. Angiography.
b. Ceftriaxone and vancomycin.
c. Nimodipine.
d. Embolization.
e. Surgical clipping.
f. Repeat the CT scan with contrast.
g. Neurosurgical consultation.

A

*B.
The number of WBCs in the CSF in this patient far exceeds the normal ratio of 1 WBC
to each 500 to 1000 RBCs. With 50,000 RBCs, there should be no more than 50 to 100 WBCs. The presence of 1250 WBCs indicates an infection, and ceftriaxone and vancomycin are the best initial therapy for bacterial meningitis. Contrast is not useful when looking for blood. Try never to answer “consultation” for anything.

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63
Q

In the USMLE when is answering “Consultation” the right answer?

A

“Consultation” is the right answer only when you want to do a particular procedure and the procedure is not given as a choice. If the right answer is “embolization” and is not listed, but “interventional neuroradiology consultation” is one of the choices, then the right answer in that case is “consultation.”

64
Q

What set of clinical features would make Anterior Spinal Artery Infarction the most likely Dx?

A
  • Loss of all function (sensory, motor and autonomic dysfunction) except for the posterior column (position [proprioception] and vibratory sensation intact)
  • Flaccid paralysis initially then after days/weeks, Spastic paralysis below the level of the infarction
  • Loss of deep tendon reflexes (DTRs) at the level of the infarction
65
Q

What set of clinical features would make Subacute Combined Degeneration of the Cord the most likely Dx?

A
  • Position and vibratory sensation are lost.
  • Spastic paralysis
  • Gait abnormalities
66
Q

What are the 2 most common causes of Subacute Combined Degeneration of the Cord?

A

From B12 deficiency or neurosyphilis.

67
Q

What are the general features of spinal trauma?

A

Acute onset of limb weakness and/or sensory disturbance below the level of the injury with the severity in proportion to the degree of injury. Sphincter function impaired. Loss of DTRs at the level of the injury followed by hyperreflexia below the level of the trauma.

68
Q

Briefly describe Brown-Sequard Syndrome

A

After unilateral hemisection of spinal cord from an injury such as a knife wound cutting half the cord or compression from a mass lesion, patients lose pain and temperature on the contralateral side from the injury, and lose motor function as well as position and vibratory sense on the ipsilateral side of the injury.

69
Q

Briefly describe Syringomyelia

A

Syringomyelia is a condition in which an abnormal fluid-filled cavity, or syrinx, develops within the central canal of the spinal cord. The syrinx is a result of disrupted CSF drainage from the central canal, commonly caused by a Chiari malformation or previous trauma to the cervical or thoracic spine

70
Q

What set of clinical features would make Syringomyelia the most likely Dx?

A

Dissociated sensory loss (loss of sensation of pain and temp while sensation of light touch, vibration, proprioception remain intact), presenting as a **cape-like distribution of decreased sensitivity to pain and temperature, and flaccid atrophic paralysis in the upper extremities.

71
Q

What is the most accurate test to Dx Syringomyelia?

A

MRI

72
Q

Briefly define a Brain Abscess and its etiology

A

A brain abscess is a collection of infected material within the parenchyma of the brain tissue acting as a space-occupying lesion. Brain abscess can spread from contiguous infection in the sinuses, mastoid air cells, or otitis media. Anything that leads to bacteremia can allow infected material to lodge in the brain. Pneumonia and endocarditis cause bacteremia, which causes brain abscess.

73
Q

What set of clinical features would make a Brain Abscess the most likely Dx?

A

headache, nausea, vomiting, fever, seizures, and focal neurological findings.

74
Q

What is the next best step after a head CT or MRI that shows a mass?

A

Biopsy. Because there is no way to distinguish a brain abscess from cancer without a biopsy. Cancer can give a fever.

75
Q

What is the best initial test when suspecting an abscess?

A

The best initial test is a head CT or MRI.

76
Q

Why is an LP contraindicated when suspecting space occupying lesion like a brain abscess or cancer?

A

Because of the possibility of herniation.

77
Q

Apart from distinguishing between a Brain Abscess or Cancer, why is a biopsy essential?

A

To determine the precise organism and its sensitivity pattern.
A precise microbiologic diagnosis is especially important given that the duration of treatment is very long (6 to 8weeks intravenously, followed by 2 to 3 more months orally).

78
Q

How would you Mgx a Brain Abscess?

A

Empiric therapy with penicillin plus metronidazole plus ceftriaxone (or cefepime) is acceptable while waiting for the results of culture. Vancomycin can be used instead of penicillin, particularly if there has been recent neurosurgery and the risk of staphylococci, especially resistant staphylococci, is greater.

79
Q

What set of clinical features would make Tuberous Sclerosis the most likely Dx?

A
  1. Neurological abnormalities: seizures, progressive psychomotor retardation, slowly progressive mental deterioration
  2. Skin:
    - Adenoma sebaceum facial angiofibroma
    - Shagreen patches (leathery plaques on the trunk)
    - Ash leaf (hypopigmented) patches
  3. Retinal lesions
80
Q

What set of clinical features would make Neurofibromatosis (von Recklinghausen Disease) the most likely Dx?

A

Neurofibromas: soft, flesh-colored lesions attached to peripheral nerves
Eighth cranial nerve tumors
Cutaneous hyperpigmented lesions (café au lait spots)
Meningioma and gliomas

81
Q

What set of clinical features would make Sturge-Weber Syndrome the most likely Dx?

A

Port-wine stain of the face
Seizures
CNS: homonymous hemianopsia, hemiparesis, mental subnormality

82
Q

Give a small briefing on Essential Tremor

A

Essential tremor occurs at both rest and with intention (reaching for things). The tremor is greatest in the hands, but can affect the head as well. The examination is otherwise normal. The tremor may affect some manual skills such as handwriting or the use of a computer keyboard. Caffeine makes it worse.

83
Q

What would make Essential Tremor the most likely Dx?

A

Tremor at rest and exertion improved with a drink of alcohol is the key to the diagnosis.

84
Q

What is the best initial Tx for Essential Tremor?

A

Propranolol

85
Q

What is Parkinsonism?

A

Parkinsonism is the loss of cells in the substantia nigra resulting in a decrease in dopamine, which leads to a significant movement disorder presenting with tremor, gait disturbance, and rigidity.

86
Q

What in the Pt’s “history” would make Parkinsonism the most likely Dx?

A

A patient age 50 to 60 or older who presents with a history of repeated head trauma from boxing or the use of antipsychotic medications such as thorazine.

87
Q

How is Parkinsonism diagnosed?

A

There is no test for parkinsonism. The diagnosis is based entirely on the clinical presentation.

88
Q

What clinical features would make Parkinsonism the most likely Dx?

A

tremor, muscular
rigidity, bradykinesia (slow movements), and a shuffling gait with
unsteadiness on turning and a tendency to fall.
Cogwheel rigidity- slowing of movement on passive flexion or extension of an extremity
Hypomimia- limited facial expression
Micrographia- small handwriting
Postural instability- orthostatic hypotension

89
Q

What distinguishes severe Parkinsonism from mild

A

Severe disease- inability to care for themselves, orthostatic

90
Q

What are the Tx options for mild Parkinsonism?

A
  1. Anticholinergic medications (benztropine and trihexyphenidyl) relieve tremor and rigidity. It is unclear why blocking acetylcholine improves symptoms of insufficient dopamine.
    - Adverse effects of dry mouth, worsening prostate hypertrophy, and constipation
  2. Amantadine may work by increasing the release of dopamine from the substantia nigra.
91
Q

What are the Tx options for severe Parkinsonism?

A
  1. Dopamine agonists: pramipexole and ropinirole are the best initial therapy in severe parkinsonism.
  2. Levodopa/carbidopa: the most effective medication. Associated with “on/off” phenomena which results in episodes of insufficient dopamine (“off”) characterized by bradykinesia. The “on” effect is too much dopamine, resulting in dyskinesia.
  3. COMT inhibitors (tolcapone, entacapone) extend the duration of levodopa/carbidopa by blocking the metabolism (catabolism) of dopamine. Used only in those treated with levodopa/carbidopa. Use when there are “on/off” phenomena to even out the dopamine level, or when the response to therapy is inadequate.
  4. MAO inhibitors (rasagiline, selegiline) as a single agent or an adjunct to levodopa/carbidopa. They block metabolism (catabolism) of dopamine.
  5. Deep brain stimulation: electrical stimulation is highly effective for tremors and rigidity in some patients.
92
Q

Why should Pt’s on MAO inhibitors avoid tyramine-containing foods (e.g., cheese)?

A

They precipitate hypertension

93
Q

Which of the following is most likely to slow the progression of parkinsonism?

a. Pramipexole.
b. Levodopa/carbidopa.
c. Rasagiline.
d. Tolcapone.
e. Amantadine.

A

*C.

Only the MAO inhibitors are associated with the possibility of retarding the progression of parkinsonism.

94
Q

A 70-year-old man with extremely severe parkinsonism comes by ambulance to the emergency department secondary to psychosis and confusion developing at home. He is maintained on levodopa/carbidopa, ropinirole, and tolcapone.

What is the most appropriate next step in management?

a. Stop levodopa/carbidopa.
b. Start clozapine.
c. Stop ropinirole.
d. Stop tolcapone.
e. Start haloperidol.

A

*B.
When a patient has very severe parkinsonism, you cannot stop medications because the patient will become “locked in” with severe bradykinesia. Psychosis and confusion are a known adverse effect of antiparkinsonian treatment. Use antipsychotic medications with the fewest extrapyramidal (antidopaminergic) effects.

95
Q

What is Lewy body dementia?

A

Parkinsonism with dementia

96
Q

What is Shy-Drager syndrome?

A

Parkinsonism predominantly with orthostasis

97
Q

What is Spasticity and what is it associated with?

How would you Mgx it?

A

Painful, contracted muscles from damage to the central nervous system is called spasticity. Spasticity is often associated with MS.
No single treatment is universally effective. Baclofen, dantrolene, and the central acting alpha agonist tizanadine may all work.

98
Q

What complaint from the Pt would make Restless Leg Syndrome the most likely Dx?

How would you Mgx it?

A

Patients report an uncomfortable sensation in the legs that is “creepy and crawly” at night. The discomfort is worsened by caffeine and relieved by moving the legs. This can happen during sleep; a patient is sometimes brought in by a bed partner who is being kicked at night.

Mgx with dopamine agonists such as pramipexole.

99
Q

What is Huntington disease (HD)?

A

It is a hereditary disease characterized by CAG trinucleotide repeat sequences on chromosome 4.

100
Q

What clinical features would make Huntington disease (HD) the most likely Dx?

A
  • Choreaform movement disorder (dyskinesia)
  • Dementia
  • Behavior changes (irritability, moodiness, antisocial behavior)
  • Onset between the ages of 30 and 50 with a family history of HD

The movement disorder of HD starts with “fidgetiness” or restlessness progressing to dystonic posturing, rigidity (no movement at all), and akinesia.

101
Q

What is the most accurate way to Dx Huntington disease (HD)?

A

Genetic analysis- CAG trinucleotide repeat sequences are found

102
Q

How would you Mgx Huntington disease (HD)?

A

Dyskinesia is treated with tetrabenazine.

Psychosis is treated with haloperidol, quetiapine, or a trial of different antipsychotics.

103
Q

What will a Head CT or MRI show in a Pt with Huntington disease (HD)?

A

Caudate nucleus involvement.

104
Q

What is Tourette Disorder (Syndrome)

A

It is a severe neurological movement disorder characterized by tics, which are involuntary, repeated, intermittent movements or vocalizations. Vocal tics- grunts and coprolalia. Motor tics (sniffing, blinking, frowning)
It is a genetic disorder that commonly presents in boys and is often associated with attention deficit hyperactivity disorder (ADHD) or obsessive-compulsive disorder (OCD).
Approximately 50% of cases resolve by adulthood.

105
Q

How would you Mgx Tourette Disorder (Syndrome)?

A
  1. Alpha-adrenergic agonists- Guanfacine (often first-line)
  2. Typical neuroleptics- Haloperidol / Pimozide
  3. Atypical neuroleptics- Risperidone (increasingly preferred because of fewer side effects)
    * Tx of associated conditions (ADHD and OCD) is intrinsic to Mgx
106
Q

What is Multiple sclerosis (MS)?

A

Multiple sclerosis (MS) is a chronic, degenerative disease of the CNS that is caused by an immune-mediated inflammatory process. This process results in the demyelination of white matter in the brain and spinal cord. MS has a higher prevalence among women and people in temperate regions such as Europe and North America.

107
Q

What clinical features would make Multiple sclerosis (MS) the most likely Dx?

A

Multiple neurological deficits of the CNS affecting any aspect of CNS functioning. The most common presentation is focal sensory symptoms, with gait and balance problems. Blurry vision or visual disturbance from optic neuritis is no longer as common as the first presentation.

108
Q

What finding on the eye exam is characteristic of Multiple sclerosis (MS)?

A

Internuclear ophthalmoplegia (INO) is the inability to adduct one eye with nystagmus in the other eye.

109
Q

What is the best initial test for Multiple sclerosis (MS)?

A

MRI is both the best initial test and the most accurate test. - MS plaques appear white and are exclusively in the white matter of the CNS

110
Q

What can a Lumbar puncture show?

A

CSF with a mild elevation in protein and fewer than 50 to 100 WBCs. Oligoclonal bands are found in about 85% of Pts. Oligoclonal bands are not specific to MS.

111
Q

How would you Mgx an acute exacerbation?

A

High-dose steroids are the best initial therapy for acute exacerbations

112
Q

How would you prevent relapse and progression of Multiple sclerosis (MS)?

A

Glatiramer and beta-interferon are the best first choice for prevention of relapse.

113
Q

What is an MRI Gadolinium Contrast Reaction?

A

A systemic overreaction to gadolinium, which results in increased collagen deposits in soft tissues and hardened fibrotic nodules develop in the skin, heart, lung, and liver. This condition, called “nephrogenic systemic fibrosis,” occurs only in renal insufficiency.

114
Q

What is Amyotrophic Lateral Sclerosis?

A

It is a neurodegenerative disease resulting in a loss of both upper and lower motor neurons.

115
Q

What clinical features would make Amyotrophic Lateral Sclerosis (ALS) the most likely Dx?

A

Weakness of unclear etiology starting in the 20s to 40s with a unique combination of upper and lower motor neuron loss. *The most serious presentation is difficulty in chewing and swallowing and a decrease in gag reflex. This leads to pooling of saliva in the pharynx and frequent episodes of aspiration.
*In ALS, there is no sensory loss and the sphincters are spared.

116
Q

What is the best initial test to Dx Amyotrophic Lateral Sclerosis (ALS)?

A

Electromyography reveals loss of neural innervation in multiple muscle groups.

117
Q

How would you Mgx Amyotrophic Lateral Sclerosis (ALS)?

A
  1. Riluzole reduces glutamate buildup in neurons and may prevent progression of disease.
  2. Baclofen treats spasticity.
  3. CPAP and BiPAP help with respiratory difficulties secondary to muscle weakness.

Tracheostomy and maintenance on a ventilator is often necessary when the disease advances

*In ALS, the most common cause of death is respiratory failure.

118
Q

What is Charcot-Marie-Tooth Disease and what are its clinical features?

A

Charcot-Marie-Tooth (CMT) is a genetic disorder with loss of both motor and sensory innervation leading to:
-Distal weakness and sensory loss
-Wasting in the legs
-Decreased deep tendon reflexes
-Tremor
Foot deformity with a high arch that does not flatten with weight bearing is common (pes cavus). The legs look like inverted champagne bottles.

119
Q

What is the most accurate test for Charcot-Marie-Tooth Disease?

A

Electromyography

120
Q

What is the most common cause of peripheral neuropathy?

What are the other causes?

A

Diabetes mellitus is the most common cause.

uremia, alcoholism, and paraproteinemias like monoclonal gammopathy of unknown significance.

121
Q

What is the best initial therapy for peripheral neuropathy?

A

pregabalin or gabapentin. Tricyclic antidepressants and most seizure medications (phenytoin, carbamazepine, lamotrigine) are effective in some people.

122
Q

What set of findings or features would make an Ulnar nerve neuropathy the most likely Dx?

A

Biker, pressure on palms of hands, trauma to the medial side of elbow.
Wasting of hypothenar eminence, pain in 4th and 5th fingers

123
Q

What set of findings or features would make a Radial nerve neuropathy the most likely Dx?

A

Pressure of inner, upper arm; falling asleep with arm over back of chair (“Saturday night palsy”); using crutches and pressure in the axilla.
Wrist drop.

124
Q

What set of findings or features would make neuropathy of the lateral cutaneous nerve of thigh/ lateral femoral cutaneous nerve the most likely Dx?

A

Obesity, pregnancy, sitting with crossed legs.

Pain/numbness of outer aspect of one thigh.

125
Q

What set of findings or features would make Tarsal tunnel / Tibial nerve neuropathy the most likely Dx?

A

Worsens with walking.

Pain/numbness in ankle and sole of foot.

126
Q

What set of findings or features would make a Peroneal nerve neuropathy the most likely Dx?

A

High boots, pressure on back of knee.

Weak foot with decreased dorsiflexion and eversion.

127
Q

What set of findings or features would make a Median nerve neuropathy the most likely Dx?

A

Typists, carpenters, working with hands.

Thenar wasting, pain/numbness in first 3 fingers.

128
Q

What are some of the causes of Facial (Seventh Cranial) Nerve Palsy or Bell Palsy?

A

Most cases of facial palsy are idiopathic. Some identified causes are Lyme disease, sarcoidosis, herpes zoster, and tumors.

129
Q

What will be the difference in presentation between Stroke and Bell Palsy?

A

Bell Palsy- Paralysis of the entire side of the face is classic. + Difficulty in closing the eye.
Stroke will paralyze only the lower half of the face because the upper half of the face receives innervation from both cerebral hemispheres.
If the patient can wrinkle her forehead on the affected side=> stroke.
If the patient cannot wrinkle his forehead on the affected side=> Bell palsy.

130
Q

Why does a Pt with Facial (Seventh Cranial) Nerve Palsy or Bell Palsy have Hyperacusis?

A

Sounds are extra loud because the seventh cranial nerve normally supplies the stapedius muscle, which acts as a “shock absorber” on the ossicles of the middle ear.

131
Q

Why does a Pt with Facial (Seventh Cranial) Nerve Palsy or Bell Palsy have Taste disturbances?

A

The seventh cranial nerve supplies the sensation of taste to the anterior two-thirds of the tongue.

132
Q

How would you Dx Facial (Seventh Cranial) Nerve Palsy or Bell Palsy?
What is the most accurate way?

A

No test is usually done because of the characteristic presentation of paralysis of half of the face.
The most accurate test is electromyography and nerve conduction studies.

133
Q

How would you Mgx Facial (Seventh Cranial) Nerve Palsy or Bell Palsy?

A

Sixty percent of patients have full recovery even without treatment. The best initial therapy is prednisone. Acyclovir is sometimes added but does not clearly help.

134
Q
A 38-year-old carpenter comes with pain near his ear that is quickly followed by weakness of one side of his face. Both the upper and lower parts of his face are weak, but sensation is intact.
What is the most common complication of his disorder?
a. Corneal ulceration.
b. Aspiration pneumonia.
c. Sinusitis.
d. Otitis media.
e. Deafness.
f. Dental caries.
A

*A.
Corneal ulceration occurs with seventh cranial nerve palsy because of difficulty in closing the eye, especially at night. This leads to dryness of the eye and ulceration. This is prevented by taping the eye shut and using lubricants in the eye. Dental caries don’t happen because although there is drooling from difficulty closing the mouth, saliva production is normal. Rather than deafness, sounds are extra loud. Aspiration does not occur because gag reflex and cough are normal.

135
Q

What is Guillain-Barré Syndrome (GBS)?

A

is an autoimmune damage of multiple peripheral nerves. By definition, there is no CNS involvement. A circulating antibody attacks the myelin sheaths of the peripheral nerves, removing their insulation. GBS is associated with Campylobacter jejuni infection.

136
Q

What set of clinical features would make Guillain-Barré Syndrome (GBS) the most likely Dx?

A

Look for weakness in the legs that ascends from the feet and moves toward the chest, associated with a loss of DTRs (deep tendon reflexes) [ankles then knees then arms].
When GBS hits the diaphragm, it is associated with respiratory muscle weakness. Autonomic dysfunction with hypotension, hypertension, or tachycardia can occur.
*Ascending weakness + loss of reflexes = GBS

137
Q

What is the most specific way to Dx Guillain-Barré Syndrome (GBS)?

What other way is there?

A

Nerve conduction studies/electromyography. These will show a decrease in the propagation of electrical impulses along the nerves, but it takes 1–2 weeks to become abnormal.

CSF shows increased protein with a normal cell count.

138
Q

What are the possible findings on a Pulmonary Function Test in a Pt with Guillain-Barré Syndrome (GBS)?

A

When the diaphragm is involved, there is a decrease in forced vital capacity and peak inspiratory pressure. Inspiration is the “active” part of breathing and the patient loses the strength to inhale.
PFTs tell who might die from GBS.

139
Q

How would you Tx Guillain-Barré Syndrome (GBS)?

A

Intravenous immunoglobulin (IVIG) or plasmapheresis, both are equal in efficacy.

  • Prednisone is a wrong answer for GBS; it does not help.
  • Combining IVIG and plasmapheresis is a wrong answer.
140
Q

A woman comes to the emergency department with bilateral leg weakness developing over the last few days. She has lost her knee jerk and ankle jerk reflexes. The weakness started in her feet and progressed up to her calves and then her thighs. She is otherwise asymptomatic.

Which of the following is the most urgent step?

a. Pulmonary function testing.
b. Arterial blood gas.
c. Nerve conduction study.
d. Lumbar puncture.
e. Peak flow meter.

A

*A.
The most dangerous thing that can happen with GBS is dysautonomia or involvement of the respiratory muscles. Peak inspiratory pressure or a decrease in forced vital capacity (FVC) is the earliest way to detect impending respiratory failure. If you wait until there is CO2 accumulation on an ABG, it is too late. Nerve conduction studies are the most accurate test, but their results are not as important as answering the question “Do you know who is going to die from respiratory failure?” Peak flow assesses expiratory function, which is not greatly impaired in GBS; peak flow is best used to assess obstructive disease such as COPD or asthma.

141
Q

What is Myasthenia Gravis (MG)?

A

It is a disorder of muscular weakness from the production of antibodies against acetylcholine receptors at the neuromuscular junction.

142
Q

What set of clinical features would make Myasthenia Gravis (MG) the most likely Dx?

A

A Pt describing “double vision and difficulty chewing,” “dysphonia,” or “weakness of limb muscles worse at the end of the day.”
This is because the extraocular muscles and mastication (maseter) are often the only 2 muscular activities universally done by people (i.e., watching TV and eating).
Physical examination reveals ptosis, weakness with sustained activity, and normal pupillary responses.
*Severe myasthenia affects respiratory muscles.

143
Q

What is the best initial test to Dx Myasthenia Gravis (MG)?

What is the most accurate test?

A

Best initial test: acetylcholine receptor antibodies (80%–90% sensitive). This is a better first answer than edrophonium testing. For patients without those antibodies, get anti-MUSK antibodies (muscle-specific kinase).

Most accurate test: Electromyography shows decreased strength with repetitive stimulation.

144
Q

Besides testing for acetylcholine receptor antibodies or anti-MUSK antibodies (muscle-specific kinase), how else can you Dx Myasthenia Gravis (MG)?

A

Edrophonium: short-acting inhibitor of acetylcholinesterase. The temporary bump up in acetylcholine levels is associated with a clear improvement in motor function that lasts for a few minutes.

145
Q

What imaging test should be done in a Pt with Myasthenia Gravis (MG)?

A

Answer: chest something. Chest x-ray, CT, or MRI are done to look for thymoma or thymic hyperplasia. CT with contrast is best.

146
Q

What is the best initial Tx for Myasthenia Gravis (MG)?

A

Best initial treatment: Neostigmine or pyridostigmine. These are longer acting versions of edrophonium.

147
Q

If Neostigmine or pyridostigmine do not control the disease. What is the most appropriate next step in management of Myasthenia Gravis (MG)?

A

A thymectomy if the patient is under age 60.
If the patient is over age 60, prednisone is used.

Azathioprine, tacrolimus, cyclophosphamide or mycophenolate are used in order to get the patient off of steroids before serious adverse effects occur. The main point is to suppress T cell function in order to control antibodies made against acetylcholine receptors.

148
Q

How would you Mgx the drooling and diarrhea adverse effects of neostigmine and pyridostigmine given to a Pt with Myasthenia Gravis (MG)?

A

Glycopyrrolate is an anticholinergic drug that blocks muscarinic receptors.
It blocks adverse effects at the muscarinic receptors of the salivary gland without blocking the nicotinic receptors at the neuromuscular junction. It also helps with COPD and decreasing oral secretions during intubation.

149
Q

How will Acute myasthenic crisis present?

How would you Mgx it?

A

Severe, overwhelming disease with profound weakness or respiratory involvement.
It is treated with IVIG or plasmapheresis.

150
Q

What is the most common cause of dementia?

A

Alzheimer disease

151
Q

Since there is no specific test for Alzheimer disease, how would you go about Dx it?

A
  1. MRI of brain- Alzheimer disease, chronic alcoholism, and untreated HIV can all give diffuse symmetrical atrophy.
  2. VDRL or RPR to exclude syphilis
  3. B12 with possible methylmalonic acid level
  4. Thyroid function test
152
Q

How would you Mgx Alzheimer disease?

A
  1. Donepezil, rivastigmine, and galantamine are equal in efficacy. All increase acetylcholine levels.
  2. Memantine
153
Q

What is Lewy Body Dementia and how is it Mgx?

A

It is dementia associated with Parkinson disease.
Treat both Parkinson disease and Alzheimer disease with levodopa/
carbidopa.

154
Q

What is Frontotemporal Dementia and how is it Mgx?

A

It is dementia in which emotional and social appropriateness are lost first.
Memory deteriorates later.
No special therapy beyond acetylcholine medications.

155
Q

What is Creutzfeldt-Jakob Disease and how is it Mgx?

A
Creutzfeldt-Jakob disease (CJD) is a neurodegenerative condition that is caused by misfolded protein particles (prions). Prion diseases are very rare overall. CJD is the most common prion disease in humans. In most cases, no direct cause of CJD can be established. However, there are also familial forms due to gene mutation or acquired forms as prion particles can be transmitted between individuals, making CJD an infectious disease. Accumulation of prion particles in the brain eventually leads to neuronal degeneration and clinical onset of the disease.
Presents with; 
-Rapidly progressive dementia
-Myoclonic jerks
-Normal head MRI or CT
-*CSF with 14-3-3 protein
-Biopsy is most accurate

No specific therapy