Neurology Pearls for the Internist Victor Sung, MD May 30, 2016

Case 1

• History: 45yo WF with MS x 20 yrs presents to ER with 2 day history of increased leg weakness, ataxia, fatigue, and urinary incontinence. At baseline she walks with a cane due to bilateral LE spastic weakness and has mild LE sensory loss. She is now unable to ambulate due to severe LE weakness

• VS: T 38.1C, BP 110/70

ARS Question

• What testing would you order first?

1. Basic labs (CBC, BMP)

2. Infectious workup (CXR, UA)

3. CT head

4. MRI brain

Case 1 Diagnostic Testing Results

• Serum chemistries, LFT’s are normal, and CBC is normal except WBC of 11,000

• Urine is cloudy and positive for nitrites and leukocyte esterase

• Chest X-ray is normal

• CT Head is almost never indicated for MS – why?

• MRI…

ARS #2

• Given these results (even without MRI), what is the most appropriate treatment for the patient?

1. Baclofen 2. Ciprofloxacin 3. Methylprednisolone 4. Prednisone 5. Glatiramer acetate

Multiple Sclerosis

• Demyelinating disease of CNS white matter characterized by neurologic dysfunction separated in time and space

• Most common presenting symptoms: optic neuritis, whole limb paresthesias, transverse myelitis

• Clinical diagnosis using McDonald Criteria (multiple discrete episodes of neurologic dysfunction = relapsing-remitting MS)

• 85% RR-MS, 15% primary progressive (PP-MS) • Supportive tests: Classic MRI brain/spine lesions; Positive

myelin basic protein (MBP) and oligoclonal bands (OCB) in CSF; Abnormal visual evoked potentials (VEP)

Multiple Sclerosis Treatment

• Acute exacerbation: • Must differentiate from pseudoexacerbation

• Most common cause of pseudoexac is UTI

• Must be debilitating symptom • Weakness, vision loss, gait problem, etc. and not just fatigue, sensory

loss

• IV methylprednisolone

• Disease-modifying therapy: ‘ABC-R’ • Injectable (decrease relapse rate by 30-40%)

• Interferons (Avonex, Betaseron, Rebif)

• Synthetic polymer glatiramer acetate (Copaxone)

• IV monthly natalizumab (Tysabri) – decreases relapse rate by 40-75%

ARS

• Given this, what would the MRI brain show?

A. Open ring-enhancing lesion

B. Periventricular white matter FLAIR lesions but no enhancement

C. Normal

CT and MRI Side by Side

• 3-D image from a large series of two-dimensional x-ray images taken around a single axis of rotation.

• Brightness = HyperDENSITY

• Bone, Blood, Bullets (or other foreign body), Contrast and Calcifications

• Darkness = HypoDENSITY

• Ischemic strokes and edema from underlying pathology (abscess or tumor)

• Contrast Uptake = Enhancement

• Metabolically active processes, breakdown of BBB (abscess or tumor)

Computed Tomography (CT)

• Brightness=HyperINTENSITY or High Signal

• Pathologic lesions, CSF

• Darkness=HypoINTENSITY or Low Signal

• CSF, underlying chronic pathology causing tissue damage.

• Contrast Uptake=Enhancement

• High degree of vascularity

• Breakdown of the blood-brain barrier

• OPEN ring enhancement: actively demyelinating lesion

• CLOSED ring enhancement: abscess, tumor

• **Note: Stroke does NOT enhance**

Magnetic Resonance Imaging (MRI)

Case 2

• 82yo WM presents to the ER after a fall at home. X-rays reveal a fractured left hip that will require surgical fixation.

• CT head radiology report:

• “No apparent tumor, stroke, or hemorrhage. Diffuse cerebral atrophy but also ventriculomegaly mildly disproportionate for age. Normal pressure hydrocephalus vs. age-related atrophy, clinical correlation recommended.”

ARS

• What do you do next?

A. MRI brain

B. Consult neurology for NPH evaluation

C. Consult neurosurgery for consideration of VP shunt

D. Just fix the hip first and defer evaluation of CT findings for outpatient

Normal Pressure Hydrocephalus (NPH)

• Clinical

• Triad: magnetic gait, urinary incontinence, cognitive impairment/dementia

• MUST have the imaging showing hydrocephalus plus the clinical

• Either gait plus incontinence or gait plus cognitive impairment or all three

• Diagnosis

• Must show clinical improvement after high volume removal of CSF

• “30/ 30 / 30” tap or lumbar CSF drain

• If no gait disorder, no way to make the diagnosis!

Normal Pressure Hydrocephalus (NPH)

Normal Pressure Hydrocephalus (NPH)

• Treatment

• VP shunt is still preferred treatment

• Good short term outcomes (80-90% improvement)

• Long term is poorer (30-90% depending on study)

• Predictors of good outcome

• Early diagnosis

• Gait disturbance as primary clinical feature

• Good initial response to high volume tap

Case 3

• 43yo WF with history of severe headaches since age 12. Frequency/severity profile was 5/1 until 10 years ago when she fell off the back of an ATV and hit the back of her head on the ground. She did not sustain LOC or other injury but was told that she had non-surgical herniated discs in her neck. Since that time, she has had essentially daily severe headache with profile 30/30 for the past 9-10 years. Her severe headaches begin from her neck and move up to involve one half and then the entire head, are throbbing, and last 8 hours or until she takes something or goes to sleep.

• For abortives, she has tried Imitrex, Zomig, Relpax, Maxalt, Axert, Frova, Fioricet, Toradol, Compazine, and opiates.

• For preventives, she has tried propranolol, ami/nortryptyline, verapamil, valproate, and topiramate.

ARS

• What would you do next for this patient?

1. MRI brain

2. MRI C-spine

3. Occipital nerve block

4. Botox injections

5. Repeat medication trials but perhaps in combination

6. Send to Pain Clinic for chronic opiates

Case 4

• 24yo WF with no significant history of headaches had 3 days of fever, malaise, increasing severity of headache. Went to OSH ER and had LP and was told was consistent with a viral syndrome and discharged. For the past 3 days has had even worse headache and has not been able to get out of bed. Headache is milder when she is lying down.

ARS

• What would you do next for this patient?

1. NS bolus x 1L

2. STAT Neurology Consult

3. Start a triptan

4. Tell patient to increase caffeine intake

5. Send to IR for blood patch

Case 5

• 32yo F reports 12 months of worsening headache and now presents with diplopia. Headache nearly constant and worse lying down – she now sleeps in recliner.

• Exam shows bilateral CN VI palsies but is otherwise unremarkable.

• Basic labs, EEG, EMG, MRI brain all unremarkable.

ARS

• What would you do next for this patient?

1. Administer tPA

2. Order MR Venogram

3. Perform LP with opening pressure

4. Blood patch

Pseudotumor Cerebri AKA Idiopathic Intracranial Hypertension (IIH)

• Classic phenotype: obese young women • Presentation

• HA increased with supine position, straining, can lead to visual obscurations, even CN deficits (IV or VI)

• Papilledema on funduscopic exam • Diagnosis

• **Elevated opening pressure on LP ** • Normal imaging (ie no actual tumor since

HA simulates headache from a tumor)

• Complication: Can lead to blindness if untreated • Treatment:

• Weight loss (5-10 pounds can be curative) • Acetazolamide, • Serial LP, or VP shunt as last resort

Other Treatments

• ER Treatment of Migraine

• Injectable triptans

• IV NSAID/anti-emetic (ie Toradol + Compazine/Reglan)

• Occipital nerve blocks

• Treatment of Status Migrainosus

• IV DHE protocols

• IV Depacon / Mag sulfate / Benadryl / Methylprednisolone / Phenobarbital

• Can be used in ER as well

Case

• 45yo WF presents with acute onset left hand clumsiness and right hemibody numbness

• MRI brain shows acute infarcts in the left thalamus, 2 in left subcortical white matter, right parietal, and right frontal, all small with no associated edema or hemorrhage

• Carotid doppler normal

• TTE shows normal LVEF, no mural thrombus, but PFO present and positive bubble study

ARS

• What do you do next?

1. LE doppler

2. Begin heparin gtt

3. Full oral anticoagulation without heparin gtt

4. Hypercoagulable workup

Ischemic Stroke Management

• IF within 3-4.5 hrs of known onset of focal symptom, can give IV tPA

• Otherwise, just give anti-platelet (ASA, clopidogrel)

• Full oral anticoagulation (ie warfarin) ONLY if Afib

• Permissive hypertension – up to 210/100 allowable for first 24 hrs

• Secondary stroke prevention

Rapid Fire Pearls

ARS

• Which of the following AED’s is NOT available for IV administration?

1. Phenytoin

2. Carbamazepine

3. Levetiracetam

4. Lacosamide

5. Valproic acid

ARS

• After a code event, presence of which of the following in the first 24 hours predicts the worst prognosis for meaningful neurologic recovery?

1. Absence of roving eye movements

2. Areflexia

3. Myoclonic jerks

4. Absence of cough/gag reflex

ARS

• What is the average duration of a generalized tonic-clonic (convulsive) seizure?

1. 10 seconds

2. 1 minute

3. 2 minutes

4. 5 minutes

5. 10 minutes

6. 15 minutes

ARS

• A 55yo patient with no significant PMH develops painless foot drop and has no sensory loss but has brisk reflexes. What should be the top item on DDx?

1. DJD of L-spine

2. MS

3. ALS

4. Parkinson’s disease

5. Myasthenia gravis

ARS

• A 60yo patient has a right hand resting tremor and was newly diagnosed with Parkinson’s disease. What is the prognosis for time to severe disability/death

1. 5-10 years

2. 10-15 years

3. 15-20 years

4. 20+ years

ARS

• What percentage of patients referred to UAB Epilepsy Monitoring Unit leave with a definitive diagnosis of pseudoseizures/conversion disorder?

1. 5-10%

2. 10-20%

3. 20-30%

4. 30-50%

5. 50-70%

ARS

• What percentage of patients with a primary complaint of “dizziness” have a primary neurologic cause?

1. 10-25%

2. 25-50%

3. 50-75%

4. >75%