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Landon Center on Aging

Neurology

Instructor: Heather Anderson, MD

 

The following 10 subjects were chosen for this web module to improve the knowledge and understanding of neurologic conditions critical to the care of geriatric patients.

Outline:

  1. Parkinson's Disease
  2. Stroke
  3. Delirium
  4. Localization
  5. Neck and back pain
  6. Sleep disturbances
  7. Cranial nerve disorders
  8. Temporal arteritis
  9. Peripheral neuropathy
  10. Dementia

 

1. PARKINSON'S DISEASE

Parkinsonism is a clinical syndrome characterized by a combination of resting tremor, rigidity, bradykinesia, and postural instability. Typically, the presence of two of these four features is necessary for the diagnosis of parkinsonism. The most common cause of parkinsonism is Parkinson’s disease (PD).  PD is a chronic, progressive neurodegenerative condition with a slow and relatively selective loss of dopaminergic neurons in the substantia nigra, so dopamine is the main neurotransmitter affected in PD.

Pathologically in PD, there is neuronal loss in the substantia nigra, especially the dopaminergic neurons in the pars compacta, causing loss of neuromelanin pigmentation. Clinical symptoms of PD occur when approximately 50-70% of these neurons are damaged.

The clinical features of PD can be categorized as motor, autonomic and neuropsychiatric symptoms.

Cardinal Motor manifestations

  • Resting tremor
    • Resting tremor is one of the most easily recognizable and the most common presentation and noticeable features of PD
    • In approximately 50% of patients, tremor is the initial symptom and approximately 15% of patients never have tremor throughout the disease course.
  • Bradykinesia
  • Rigidity
  • In later disease stages postural instability
    • Postural instability associated with propulsion and retropulsion is one of the most disabling symptoms of PD

Autonomic dysfunction

  • Occur in approximately 30% of the PD patients and can occur in all stages of PD
  • Autonomic dysfunction early in the disease course, generally suggests Shy Drager syndrome (a form of multiple system atrophy) – see below (MSA in “Parkinson’s Plus” Syndromes list)
  • Autonomic symptoms seen in PD include
    • Orthostatic hypotension
    • Constipation
    • Dysphagia
    • Excessive sweating
    • Heat intolerance
    • Urinary
    • Sexual dysfunction.

Neuropsychiatric symptoms in PD

  • Dementia
    • Estimated to occur in 30-40% of PD patients.
  • Depression
    • Prevalence of depression in PD is about 31%.
  • Anxiety
    • Anxiety has been reported in about 38% of PD patients.
  • Psychosis
    • Can occur in PD patients on anti-PD medications
    • Rarely seen in untreated PD patients
    • Psychotic symptoms in PD include
      • Hallucinations – the most common psychotic symptom, and these are usually visual
      • Delusions (paranoia) and delirium are less common

Motor complications due to levodopa and disease

  • Freezing of gait
    • Freezing consists of a sudden, transient inability to move as if the feet are glued to the ground
  • Levodopa induced motor fluctuations including end of dose wearing off, on-off episodes, early morning akinesia
  • Dyskinesia including peak dose dyskinesia, biphasic dyskinesia and end of dose dystonia
“Parkinson’s Plus” Syndromes
  • Progressive supranuclear palsy (PSP)
    • Gait instability, rigidity, no voluntary vertical eye movements but preserved Doll’s eyes
  • Multiple system atrophy (MSA)
    • Shy Drager syndrome – orthostatic hypotension, syncope, autonomic dysfunction, ataxia
      • Motor manifestations become less responsive to levodopa as a general rule in Shy Drager syndrome
  • Corticobasal ganglionic degeneration – alien hand syndrome, apraxia, dysphasia, cortical sensory loss
  • Dementia with Lewy bodies
    • Fluctuations in cognitive function, hallucinations, parkinsonism, falls, syncope, sensitivity to neuroleptics making parkinsonism worse

To view this movie clip be sure that your browser popup blocker is turned off to allow popups.

  • Parkinsonian Gait Demonstration - This man's gait is bradykinetic and his steps are smaller than usual. There is also the pill-rolling tremor in his hands. He turns en bloc, and there is decreased facial expression

The above movie drawn from the Neurologic Exam and PediNeurologic Exam websites is used by permission of Paul D. Larsen, M.D., University of Nebraska Medical Center and Suzanne S. Stensaas, Ph.D., University of Utah School of Medicine. Additional materials for Neurologic Exam are drawn from resources provided by Alejandro Stern, Stern Foundation, Buenos Aires, Argentina; Kathleen Digre, M.D., University of Utah; and Daniel Jacobson, M.D., Marshfield Clinic, Wisconsin. Subsequent re-use of any materials outside of this program, presentation, or website requires permission from the original producers.


CASE

A 65 year-old right-handed white male with a 3-year history of tremors in his hands when he is sitting, watching TV. The tremor started on the left initially, but now there is a slight tremor on the right. His wife reports that he is walking more slowly and shuffles his feet.  His handwriting has also gotten smaller.

Neurologic Examination:

  • Mental Status: Slow to answer. Hypophonic voice.
  • Cranial nerves: Masked face. Normal extraocular movement. All other cranial nerves are intact.
  • Motor: Resting, pill-rolling tremor, cogwheel rigidity in the arms more than the legs, normal strength
  • Sensory: Normal sensory exam
  • Reflexes: Normal reflexes
  • Cerebellum: No tremor on finger-to-nose testing, and no evidence of dysmetria.
  • Gait: Slightly slow gait with reduced arm swing, multi-step turn, and obvious tremor while walking.

Question: What is the diagnosis?

What if this same patient is/has: Click on the correct answer

  1. Falling A LOT with inability to voluntarily look down, but could overcome this with Doll’s eyes maneuver.
    1. Cortical basal ganglionic degeneration
    2. Dementia with Lewy Bodies
    3. Progressive Supranuclear Palsy
    4. Shy Drager Syndrome

  2. Passing out
    1. Cortical basal ganglionic degeneration
    2. Dementia with Lewy Bodies
    3. Progressive Supranuclear Palsy
    4. Shy Drager Syndrome

  3. One arm has a mind of its own
    1. Cortical basal ganglionic degeneration
    2. Dementia with Lewy Bodies
    3. Progressive Supranuclear Palsy
    4. Shy Drager Syndrome

  4. Dementia is rather prominent as compared to motor symptoms, and cognitive dysfunction began within a year of onset of motor symptoms
    1. Cortical basal ganglionic degeneration
    2. Dementia with Lewy Bodies
    3. Progressive Supranuclear Palsy
    4. Shy Drager Syndrome

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2. STROKE

A stroke occurs when there is ischemia to part of the brain either through an ischemic or hemorrhagic event

  • Ischemic strokes – accounts for ~85% of strokes

    Ischemic strokes - accounts for ~85% of strokes
    Source: tinyurl.com/m696kl

    • Thrombotic
      • Blood clot, ruptured plaque or thrombus forms in a cerebral artery and occludes an intracerebral artery
      • Gradual progression of symptoms
    • Embolic
      • Blood clot forms elsewhere in the body, travels to the cerebral vasculature, and occludes an intracerebral artery
      • Rapid onset of symptoms because of the artery
      • Usually cardiac source, but could be from carotids or vertebral arteries, or paradoxical emboli from a DVT in the setting of patent foramen ovale (PFO)

  • Hemorrhagic stroke – accounts for ~15% of strokes

    Hemorrhagic stroke - accounts for ~15% of strokes
    Source: www.emedicine.com/emerg/topic557.htm

    • Leakage from small intracerebral arteries into the brain parenchyma
    • Most common cause is chronic hypertension leading to damage of the small intracerebral arteries
    • Most common sites for hemorrhagic stroke
      • Thalamus
      • Putamen
      • Cerebelleum
      • Brainstem
    • Symptoms often accompanied by sudden and severe headache, nausea and vomiting
    • Seizures are more common in hemorrhagic than in ischemic strokes and can occur in up to 28% of hemorrhagic strokes
    • Hemorrhagic strokes are not to be confused with hemorrhagic transformation of ischemic strokes which are quite common and usually asymptomatic

Stroke symptoms – Acute onset of neurologic deficits

  • Hemiparesis
  • Hemisensory loss
  • Monocular or binocular visual loss
  • Visual field deficits
  • Diplopia
  • Dysarthria
  • Ataxia
  • Vertigo
  • Aphasia (expressive or receptive)

Stroke risk factors

  • Age over 55
  • Male gender
  • African-American
  • Diabetes
  • Family history of stroke
  • Previous stroke or TIA
  • High cholesterol
  • Hypertension
  • Heart disease (coronary artery disease, left ventricular hypertrophy, chronic atrial fibrillation)
  • Smoking

Evaluation of a stroke patient

  • Time is of the essence – the one and only one treatment for a stroke is tPA (tissue plasminogen activator)
    • 3 hour window for administering IV tPA
    • 6 hour window for administering intraarterial tPA (angiogram with tPA injected right at the site of the clot)
  • CT head without contrast
    • Looking for hemorrhage – if there is already a hemorrhage, not a tPA candidate
    • DO NOT USE THE CT TO DIAGNOSE THE STROKE – Use your clinical history and exam to diagnose the stroke.  A CT that already shows a stroke or edema soon after the stroke occurred indicates a large stroke in which there is an increased risk of hemorrhage with tPA.  A CT which is entirely clear, with no infarct or edema or hemorrhage, is the PERFECT situation in which to give tPA.
  • Labs
    • Fasting lipids
    • Fasting blood sugar
    • Reserve hypercoagulable work-up for occasion in which no obvious cause of the stroke can be determined (usually performed in young stroke patients)
    • Coagulation studies is most helpful if the patient is on anticoagulants
    • Urine drug screen in selected patients
  • Tests
    • EKG – rule-out atrial fibrillation
    • Echocardiogram – rule-out cardiac source of emboli, determine ejection fraction
    • Carotid ultrasound - Only if the stroke is anterior circulation. If it arose from the posterior circulation, the presence of carotid stenosis is irrelevant since it supplies the anterior circulation. In this situation, MRA (MR angiogram) would be better.
    • Only if the stroke is anterior circulation.  If it arose from the posterior circulation, the presence of carotid stenosis is irrelevant since it supplies the anterior circulation.  In this situation, MRA (MR angiogram) would be better.
    • MRI brain once the patient is stable

Treatment

  • tPA if the patient is a candidate
    • Implement antiplatelet therapy for secondary stroke prevention, not generally anticoagulation.  Heparin just yields increased risk of hemorrhage but no increased benefit after most strokes.
    • The American Heart Associate evidence-based guidelines consider ASA alone, Plavix (clopidogrel) or Aggrenox (dipyridamole/aspirin) all reasonable options as first line therapy for secondary stroke prevention.
  • Liberal management of hypertension
    • Chronic hypertension is a risk factor for stroke, however in the setting of an acute stroke, hypoperfusion makes stroke symptoms worse so liberal management of hypertension is best
  • Aggressive treatment of diabetes
  • Initiate statin for cholesterol management
  • Anticoagulation in only selected cases (atrial fibrillation, low ejection fraction, anterior wall aneurysm, or carotid or vertebral dissection)
  • Physical therapy, occupational therapy, and speech therapy

CASE

A 70 year-old right handed white male awoke with right-sided weakness and expressive aphasia.  He presented to the ER within 45 minutes of discovering the weakness.  He has never had a similar event.

Physical Examination:

  • Mental status: Awake, alert, and oriented.  His comprehension is intact, but he is having difficulty expressing himself with word-finding difficulty.
  • Cranial nerves: Right lower facial weakness.  Eyes deviated to the left.  Right superior quadrantanopia.  All other cranial nerves are intact.
  • Motor: Dense right-sided hemiplegia.  Strength is normal on the left.
  • Sensory: Diminished sensation in right face, arm, and leg.
  • Reflexes: 2/4 on the left, 3/4 on the right.  Toes are downgoing on the left and upgoing on the right.
  • Cerebellum: Unable to perform finger-to-nose or heel-to-shin testing on the right.
  • Gait: Unable to ambulate.

Evaluation:

CT head without contrast is negative for hemorrhage, edema, and infarct.

Question: What is your recommendation to your attending - Should you give the patient TPA? (Click on your choice to see the answers)

Question: What labs should you draw?

Question: What tests should you order?

Question: Assume the patient had slurred speech, trouble swallowing, double vision, and ataxia instead of right-sided weakness, would you still get a carotid Doppler?

Question: The patient is not a TPA candidate, but he has had a big stroke and has major deficits.  We should start heparin, right?

Question: If you chose not to anticoagulate, what do we give then?

Question: In what situations would a stroke patient be placed on anticoagulation (heparin, Coumadin)?

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3. DELIRIUM

Delirium, or mental status change, is very frequent, and very elderly patients and those with preexisting cognitive changes are especially susceptible to prolonged cognitive decline following an acute event. 

Evaluation

  • Determine the baseline cognitive function of the patient.  Does the patient have preexisting dementia or depression or other psychiatric diagnoses?
  • What was the course of the presentation? 
    • An acute change in mental status from baseline is not consistent with dementia and suggests delirium.
    • A rapidly fluctuating course (over minutes to hours) and an abnormal level of consciousness are also highly suggestive of delirium
  • Delirium is diagnosed with the use of the Confusion Assessment Method (CAM)
    • Sensitivity 94%-100%
    • Specificity 90%-95%

    Reference: Inouye, et.al. Ann Intern Med 1990;113:941-948

  • In critically ill patients, on and off the ventilator, who are not able to speak, the CAM-ICU is used

    References:
    Inouye, et al. Ann Intern Med 1990:113:941-948
    Ely, et. al. CCM 2001;29:1370-1379
    Ely, et. al. JAMA 2001; 286:2703-2710

     

Confusion Assessment Method (CAM)

**A diagnosis of delirium is made with Items 1 and 2 and either items 3 or 4 present**

  1. Acute onset and fluctuating course
    • Acute change in mental status from baseline
      • Dementia syndromes typically develop over months to years rather than acutely.
    • Rapidly fluctuating course (over minutes to hours)
      • In delirium, confusion comes and goes or increases and decreases in severity

                                          AND

  1. Inattention
    • Inability to stay focused
    • Easily distractable
    • Difficulty tracking conversation

    AND EITHER

  2. Disorganized thinking
    • Rambling or irrelevant conversation
    • Unclear or illogical flow of ideas
    • Unpredictable switching from subject to subject

    OR

  3. Altered level of consciousness (any of the following count as altered level of consciousness)
    • Vigilant
      • Hyperalert
      • Overly sensitive to environmental stimuli
      • Startled very easily
    • Lethargic
      • Drowsy but easily aroused
    • Stupor
      • Difficult to arouse
    • Coma
    • Unarousable

CAM-ICU for critically ill patients

**A diagnosis of delirium is made with Items 1 and 2 and either items 3 or 4 present**

  1. Acute onset OR fluctuating course (as in CAM above)

    AND

  2. Inattention using the Letters Attention Test – More than 2 errors is considered abnormal
    • Directions: Say to the patient, “I am going to read you a series of 10 letters.  Whenever you hear the letter ‘A,’ indicate by squeezing my hand.”  Read letters from the following letter list in a normal tone 3 seconds apart:

    S A V E A H A A R T

    Errors are counted when patient fails to squeeze on the letter “A” and when the patient squeezes on any letter other than “A.”

    AND EITHER

  3. Altered level of consciousness
    • Any level of consciousness other than alert and calm

    OR

  4. Disorganized thinking – Combined number of errors >1 is considered abnormal
    • Yes/No Questions
      • Will a stone float on water?
      • Are there fish in the sea?
      • Does one pound weigh more than two pounds?
      • Can you use a hammer to pound a nail? 

                                          Errors are counted when the patient incorrectly answers a question.  

    •  Command  
      • Say to patient:  “Hold up this many fingers” (Hold 2 fingers in front of patient)   “Now do the same thing with the other hand” (Do not repeat number of fingers)   *If pt is unable to move both arms, for 2nd part of command ask patient to “Add one more finger”

      An error is counted if patient is unable to complete the entire command.

Risk factors

  • Predisposing factors
    • Advanced age
    • Preexisting dementia
    • Preexisting functional impairment in activities of daily living
    • High medical comorbidity
    • Sensory impairment (poor vision and hearing)
    • History of alcohol abuse
  • Acute precipitating factors
    • Medications, especially those that are sedating or highly anticholinergic
    • Infections
      • Pneumonia
      • Urinary tract infection
      • Infected decubitus ulcer
    • Uncontrolled pain
    • Low hematocrit level
    • Bed rest
    • Use of certain indwelling devices and restraints

Mnemonic for Reversible Causes of Delirium (Geriatrics Review Syllabus, 6th ed.)

Drugs

Any new additions, increased doses, or interactions

Consider over-the-counter drugs and alcohol

Consider esp. high-risk drugs (anticholingerics, tricyclic antidepressants, some opioids

Electrolyte disturbances

Especially dehydration, sodium imbalance

Thyroid abnormalities

Lack of drugs

Withdrawals from chronically used sedatives, including alcohol and sleeping pills

Poorly controlled pain (lack of analgesia)

Infection Especially urinary and respiratory tract infections
Reduced sensory input Poor vision, poor hearing
Intracranial

Infection, hemorrhage, stroke, tumor

Rare: consider only if new focal neurologic findings, suggestive history, or work-up otherwise negative

Urinary, fecal

Urinary retention: “cystocerebral syndrome”

Fecal impaction

Myocardial, pulmonary Myocardial infarction, arrhythmia, exacerbation of heart failure, exacerbation of chronic obstructive pulmonary disease, hypoxia

CASE

An 85 year-old woman is brought by her family to the doctor with concern about increasing confusion and agitation over the last day.  Her exam is significant for decreased responsiveness, restlessness, and has an odor of urine.  There is no focal neurologic deficit. (Click on questions to see the answers)

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4. LOCALIZATION


Source: Image adapted from an image at coachhouserehab.com/images/brain.jpg

Frontal lobe

  • Contralateral weakness due to involvement in the motor strip (precentral gyrus)
  • Urinary incontinence when bilateral lesions are present because the micturition center is located in the frontal lobes
  • Expressive aphasia (Broca’s aphasia) involves a lesion in the dominant posterior inferior frontal region (Brodmann’s area 44 and 45)
  • Executive dysfunction and personality changes when the prefrontal cortex is affected

Parietal lobe

  • Contralateral sensory loss due to involvement in the post-central gyrus
  • Inferior quadrantanopia – caused by a lesion along the fibers from the superior retina (supplying the lower visual field)


Localization in Clinical Neurology 4th ed. Brazis et al.

  • Apraxia (dressing, construction, ideomotor) – disorder of skilled movement, not caused by weakness or sensory loss, occur with a lesion in the dominant inferior parietal lobe
  • Extinction or simultanagnosia on double simultaneous stimulation occurs with lesions in the left parietal lobe.  The individual can sense light touch in both arms, for example, individually, however when both arms are touched simultaneously, only 1 side is felt. Occurs with lesions in the left parietal lobe.
  • Neglect - inability to report, respond, or orient to stimuli, generally in the contralesional space. Occurs with a lesions in the right parietal lobe
  • Anosagnosia (denial of deficits).  The patient clearly has weakness on one side but the patient denies having this deficit occurs with a lesions in the right parietal lobe.
  • Gerstmann’s syndrome occurs with a lesion in the left parietal lobe.
    • Finger agnosia - difficulty naming and differentiating among the fingers of either hand as well as the hands of others
    • Agraphia (inability to draw)
    • Acalculia (inability to calculate)
    • Right-left disorientation
  • Balint’s syndrome occurs with bilateral parietal lobe lesions
    • Inability to voluntarily control the gaze (ocular apraxia)
    • Inability to integrate components of a visual scene (simultanagnosia)
    • Inability to accurately reach for an object with visual guidance (optic ataxia)
Temporal lobe
  • Receptive aphasia (Wernicke’s aphasia) - Brodmann's area 22 occurs with a lesion in the dominant laterosuperior temporal lobe
  • Sensory amusia (inability to interpret or appreciate musical sounds) occurs with a lesion in the nondominant laterosuperior temporal lobe
  • Sensory aprosodia (imparied ability to comprehend the emotion conveyed in spoken language and impairment of identification of emotional gesturing) occurs with a lesion in the nondominant laterosuperior temporal lobe
  • Superior quadrantanopia or “pie-in-the-sky defect” is caused by a lesion along the fibers from the inferior retina (supplying the upper visual field) as they travel through Meyer’s loop


    Localization in Clinical Neurology 4th ed. Brazis et al.

    (Click here for picture)
  • Kluver-Bucy syndrome occurs with lesions affecting the tips of the bitemporal lobes
    • hyperorality, hypersexuality, flattened emotions, memory loss, inability to recognize faces and objects
  • Amnesia can occur when the inferomedial aspect of the temporal lobes are involved, affecting the amygdala and hippocampus (the “memory centers” of the brain)
  • Impaired recognition of facial emotional expression occurs with lesions in the nondominant lateroinferior temporal lobe

Occipital lobe

  • Homonymous hemianopsia (loss of entire half of visual field).


    Localization in Clinical Neurology 4th ed. Brazis et al.

  • Macular sparing is present because this is a watershed area, supplied by terminal branches of the Posterior Cerebral Artery (PCA) and Middle Cerebral Artery (MCA).  Since occipital lobe strokes are due to PCA occlusion, the macula is spared because of supply by the MCA.

Brainstem

  • Wallenberg syndrome-lesion of lateral medulla
    • Loss of pain and temperature on ipsilateral face and contralateral limbs and trunk
    • Loss of vibrations, proprioception, ataxia in ipsilateral limbs
    • Ipsilateral Homer's syndrome, vertigo, nystagmus, hoarseness, and dysphagia are often present

Cerebral Artery Territories

  • middle cerebral artery (pink)
  • posterior cerebral artery (green)
  • anterior cerebral artery (blue)

http://www.cnsforum.com/content/pictures/imagebank/hirespng/brain_struc_stroke.png

Deficits can be due to tumors, etc as well as from strokes.  If a deficit is due to a stroke, here is a table summarizing the localization and relevant arterial distribution.  For more detail on the deficits, please refer to the text below.

Anterior Cerebral Artery

Middle Cerebral Artery

Posterior Cerebral Artery

  • Rare (0.6-3%)
  • Contralateral weakness affecting leg > arm
  • May have lack of initiation or abulia (lack of will or motivation)
  • May have sensory loss to contralateral leg
  • May have gait and postural disorders
  • Most common site of ischemic stroke
  • Contralateral weakness affecting face/arm > leg
  • May have deviation of the eyes to the side of the lesion
  • Dominant hemisphere: Broca’s, Wernicke’s, conduction, or global aphasia
  • May have Gerstmann syndrome (see below)
  • Nondominant hemisphere: inattention, neglect, denial, apraxia
  • Contralateral homonymous hemianopsia with macular sparing (see below)

 

CASE

The following deficits are found on physical examination of a stroke patient.  Based on your knowledge of the neurologic examination and physiology, a lesion in what area of the brain is the most likely to cause these deficits?

  1. Loss of pain, temperature on ipsilateral face and contralateral body, loss of vibration, proprioception ipsilateral limbs
    1. Frontal lobe
    2. Parietal lobe
    3. Temporal lobe
    4. Occipital lobe
    5. Brainstem (lateral medullary syndrome)

  2. Contralateral sensory loss, inferior quadrantanopia (loss of vision in the right lower quadrant, for example), apraxia
    1. Frontal lobe
    2. Parietal lobe
    3. Temporal lobe
    4. Occipital lobe
    5. Brainstem (lateral medullary syndrome)

  3. Homonymous hemianopsia (loss of right half of the visual field of both eyes, for example)
    1. Frontal lobe
    2. Parietal lobe
    3. Temporal lobe
    4. Occipital lobe
    5. Brainstem (lateral medullary syndrome)

  4. Receptive aphasia, superior quadrantanopia (pie-in-the-sky defect)
    1. Frontal lobe
    2. Parietal lobe
    3. Temporal lobe
    4. Occipital lobe
    5. Brainstem (lateral medullary syndrome)

  5. Contralateral weakness, expressive aphasia
    1. Frontal lobe
    2. Parietal lobe
    3. Temporal lobe
    4. Occipital lobe
    5. Brainstem (lateral medullary syndrome)

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5. NECK AND BACK PAIN

When a patient comes in complaining of neck or back pain, it is important to ask them to describe the pain in as much detail as possible.  Ask them if the pain is just located in the neck or back or does it radiate or shoot down a limb. Ask them to point with a finger and show the line of radiation (Click here for "Dermatone Map of the Body"). This will help you localize the pain.  Have them hold the arm in the anatomic position, and if the pain radiates into the thumb, then you need to think about a C6 radiculopathy.  Also, ask about the characteristics of the pain (sharp, aching, shooting, electrical, burning, tingling).  This helps you differentiate the etiology of the pain.  If the pain is aching or over a joint, then it is probably musculoskeletal.  If it is neuropathic in description (electrical, burning, tingling), then you need to think about a pinched nerve, etc.

Radiculopathy

What is it? Click here for picture

  • Nerve root becomes compressed, usually because of a herniated disc or degenerative disc disease
  • Causes pain, weakness, sensory loss, and reflex asymmetry in the distribution of that nerve root

Complications

  • Disc herniation may not just entrap a nerve root, but may cause spinal stenosis and pressure on the spinal cord (Click here for picture)
    • Ask every radiculopathy patient if there has been any change in bowel/bladder or any gait changes.  If so, this indicates the presence of spinal stenosis

The neuro exam will help you localize the lesion. 

  • Myotomal distribution
    • Elbow flexion (biceps) is supplied by C5-6
    • Elbow and finger and wrist extension – C7
    • Finger flexion – C8-T1
    • Hip flexion – L3
    • Knee extension – L4
    • Knee flexion – L5
    • Ankle dorsiflexion – L5
    • Ankle plantarflexion – S1
  • Check pinprick over the dermatomal distributions
    • For the arm, I would check this in the lateral aspect of the arm (C5), thumb (C6), middle finger (C7), little finger (C8), and medial aspect of the arm (T1)
    • For the leg, I would check this over the medial aspect of the foot (L5) and lateral aspect of the foot (S1).  Remember the big toe is bigger than the little toe, and 5 is bigger than 1, therefore, L5 innervates the medial aspect of the foot, and S1 innervates the lateral aspect.
  • Check reflexes
    • Biceps and brachioradialis are supplied by C5-6
    • Triceps is supplied by C7
    • Knee jerk is supplied by L2,3,4
    • Ankle jerk is supplied by S1
    • There is no abnormal Babinski (upgoing toes) with an isolated radiculopathy – Upgoing toes indicates an upper motor neuron lesion such as spinal stenosis.

The most common nerve root to get compressed in the neck is C7 (60%) with C6 being the 2nd most common (25%).  The most common nerve roots to get compressed in the lumbar spine are L5 and S1.

Pain originating in the neck or back and “radiating” down the respective limb often represents radiculopathy (nerve root impingement), but not necessarily.  If there is normal strength, sensation, and reflexes, then you need to think about hip or other joint pathology.

  • Lumbar radiculopathy
    • Positive straight leg raise
    • Increased pain with increased abdominal pressure (coughing, sneezing, bearing down)
    • Weakness or sensory or reflex changes in L5 or S1 distribution
  • Hip pathology
    • Increased pain when lying on the affected side
    • Point tenderness
    • Normal sensation, strength, and reflexes

Spinal Stenosis

Hyperreflexia indicates an upper motor neuron lesion at least 1 level above the level of the root supplying the reflex.  If a patient has hyperreflexia in both legs with normal reflexes in the arms, the lesion must be above L4 which supplies the knee jerks.  The upper motor neuron lesion could be in the upper lumbar spine (very uncommon), in the thoracic spine (exceedingly uncommon), or in the cervical spine (much more likely).  So, if you must image a spinal level for a patient with hyperreflexia in the legs and normal reflexes in the arms, start with an MRI of the cervical spine.  Perhaps the cervical stenosis is not high enough in the cervical spine to affect the reflexes in the arms.  Hyperreflexia in the arms AND legs probably indicates an upper cervical lesion or perhaps a bilateral lesion in the brain.

Spinal Cord Injury

Treatment

  • Surgical stabilization
  • Steroids (IV Solumedrol (methylprednisolone)) to prevent further neurologic damage by reducing edema
    • Edema in a fixed, rigid column presses normal spinal tissue against the vertebral bodies, causing more neurologic damage

For a patient with bilateral leg weakness and low back pain, be sure to ask about urinary changes (incontinence and retention) and saddle anesthesia.  If these are present, you need to check for anal sphincter tone.  If this is reduced, the patient needs an emergent MRI of the lumbar spine to rule out cauda equina syndrome.

CASE 1

A 34 year-old man presents with gait difficulty.  On examination, he is found to have normal reflexes and strength in his arms and hyperreflexia in his legs with upgoing toes, and his gait is spastic.

Question: If you were to MRI the spine of this patient with normal reflexes in the arms and hyperreflexia in the legs, which level would you MRI first? (Click on your choice to see the answers)

  1. Cervical spine
  2. Thoracic spine
  3. Lumbar spine

CASE 2

A 45 year-old man presents with neck pain shooting into the lateral aspect of his left arm, into the thumb.  He is also having weakness in his left arm when carrying things.  On examination, he has weakness of elbow flexion on the left with diminished biceps and brachioradialis reflexes but triceps reflex is normal on the left.  Pinprick is diminished over the lateral aspect of his left forearm and hand.

Question: Which cervical nerve root is getting compressed?

  1. C5
  2. C6
  3. C7
  4. C8
  5. T1

Question: Upon further examination, he is found to have hyperreflexia in his legs with upgoing toes, and he is complaining of urinary urgency and retention. (Click on question to see the answers)

  1. What is the explanation for these additional exam findings?

CASE 3

67 year-old woman with pain radiating down the lateral aspect of the right leg to the midcalf.  Strength, reflexes, and sensation are all normal.  There is point tenderness over the lateral aspect of the right hip.

Question: Where is the lesion – right hip or lumbar radiculopathy?

  1. Right hip
  2. Lumbar radiculopathy

 

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6. SLEEP DISTURBANCES

The most common sleep disturbance is insufficient sleep syndrome (sleep deprivation) followed closely by sleep apnea

Neurologic causes of sleep disturbances

  • Narcolepsy
    • Symptoms
      • Cataplexy – loss of muscle tone, not loss of consciousness, in response to strong emotional stimuli (laughter, startle)
      • Excessive daytime sleepiness – Narcoleptics fall asleep in dangerous or socially inappropriate situations (while driving, while teaching class, or during sexual intercourse for example)
      • Hypnagogic hallucination - episodes of seeing and hearing things as one is falling asleep
      • Hypnopompic hallucinations – vivid dreamlike hallucination that occurs as one is waking up
      • Sleep paralysis – temporary paralysis of the body shortly after waking up when the brain awakens from a REM state, but the bodily paralysis persists
    • Diagnosis
      • Multiple sleep latency test (MSLT) – a series of 5 nap opportunities are recorded where the time to drop into REM sleep is measured.  Sleep-onset REM (SOREM) is diagnostic of narcolepsy
    • Treatment
      • Avoid alcohol and drug use, which may exacerbate symptoms
      • CNS stimulants
        • Ritalin (methylphenidate)
        • Provigil (modafinil)
      • Anticataplectic agents
        • Anafranil (clomipramine)
        • Prozac (fluoxetine)
        • Xyrem (gamma hydroxybutyrate (GHB))
  • Restless legs syndrome
    • Symptoms
      • Difficulty getting into a comfortable position when trying to go to sleep
      • Antsy, creepy-crawly sensation in the legs
      • Symptoms are temporarily relieved by walking or repositioning
    • Differential diagnosis
      • Iron deficiency
      • Peripheral neuropathy
    • Treatment
      • Benzodiazepines
      • Levodopa or dopamine agonists such as Mirapex (pramipexole) or Requip (ropinirole)
      • Opioids
      • Neurontin (gabapentin)
  • Periodic limb movement disorder
    • Symptoms - stereotyped periodic limb movements during sleep that cause awakening during the night
    • Treatment
      • Benzodiazepines
      • Levodopa or dopamine agonists such as Mirapex (pramipexole) or Requip (ropinirole)
      • Neurontin (gabapentin)
      • Baclofen (lioresal)

CASE

67 year-old woman with a couple of year history of difficulty falling asleep due to discomfort in her legs.  She describes this as an antsy sensation, and she needs to keep moving around, trying to find a comfortable position.  Her examination is significant for diminished vibration and proprioception in the feet and diminished ankle jerks but normal neurologic examination otherwise. (Click on questions to see the answers)

  1. What is the diagnosis of the sleep disorder?
  2. What tests would you order to determine treatable causes of this condition?
  3. What classes of medications or names of medications can improve these symptoms?

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7. CRANIAL NERVE DISORDERS

Cranial Nerve I – Olfactory nerve

  • Head injury/shearing of the olfactory nerves at the cribriform plate
  • Diminished olfaction in Parkinson’s disease and Alzheimer’s disease

Cranial Nerve II – Optic nerve

  • Evaluation
    • Visual acuity using the Snellen eye chart
    • Visual field testing
    • Light reflex (CN II is the afferent and CN III is the efferent limb of the reflex)
    • Fundoscopic examination
  • Differential diagnosis
    • Optic neuritis
    • Papilledema – pressure on the optic nerve from increased intracranial pressure causes enlargement of the physiologic blind spot and can ultimately cause blindness (Click here for Figure)

Cranial Nerve III – Oculomotor nerve

  • Evaluation
    • Supplies all of the “H” test except for SO4LR6 (superior oblique is supplied by CN IV and the lateral rectus is supplied by CN VI)
  • Injury
    • Results in diplopia
    • Mild ptosis of the ipsilateral eye, failure of the eye to adduct and impaired upgaze
    • If the parasympathetic fibers which surround CN III are affected, the pupil will be affected
      • Pupillary sparing left CN III palsy (Click here for Picture) is likely due to an infarct of CN III.  Note that there is ptosis on the left (so examiner is holding open the left eye), left eye fails to adduct, and pupils are the same size.
      • Pupillary involving left CN III palsy is likely due to a compressive lesion such as a mass or aneurysm.  Note that the patient is attempting to fixate on an object slightly to the right.  There is mild ptosis on the left (so examiner is holding open the left eye), the left eye fails to adduct, and the left pupils fails to constrict and is larger than the right.

Cranial Nerve IV – Trochlear nerve

  • Evaluation
    • Depression of the eye primarily on adduction
  • Injury
    • Causes diplopia which is better when tilting the head to the contralateral side, and the diplopia is worse when tilting to the ipsilateral side.
      • Example: Left CN IV palsy – diplopia is better when tilting the head to the right and worse when tilting the head to the left

Cranial Nerve V – Trigeminal nerve

  • Evaluation
    • Assess light touch in the V1, V2, and V3 distributions on the face
    • Feel the bulk of the masseter muscle when clenching the teeth.  The muscles of mastication are supplied by V3.
    • Corneal reflex (CN V is the afferent and CN VII is the efferent limb of the reflex)
  • Injury
    • Sensory loss in the affected distribution
    • Weakness of mastication on the affected side if V3 is affected
    • Trigeminal neuralgia – brief, intense lancinating pain in 1 or 2 trigeminal distributions provoked by wind, touch, cold, eating, brushing teeth, etc.
      • Treatment = carbamazepine

Cranial Nerve VI – Abducens nerve

  • Evaluation
    • Abduction of the eye
  • Injury
    • Causes diplopia which is better when looking to the contralateral side and worse when looking to the ipsilateral side.

Cranial Nerve VII – Facial nerve

  • Evaluation
    • Muscles of facial expression
  • Injury
    • Ipsilateral facial weakness
      • Central CN VII palsy – Weakness of the lower face on the contralateral side sparing the upper face because portion of the 7th nerve nucleus that supplies the upper face receives bilateral corticobulbar (upper motor neuron) input.
      • Peripheral CN VII palsy – Weakness of the entire ipsilateral side of the face, both upper and lower face.  You can have as much dual innervation as possible, but if the final common pathway is damaged, the upper face is still going to be weak.
  • Differential diagnosis of peripheral CN palsy
    • Bell’s palsy – ipsilateral upper and lower facial weakness
    • Ramsey-Hunt Syndrome – Bell’s palsy plus vesicles in the ipsilateral ear caused by Herpes Zoster.  Treatment is Acyclovir

Cranial Nerve VIII – Vestibulocochlear nerve

  • Evaluation
    • Acoustic component
      • Whisper or finger rub or clicking next to each ear
    • Vestibular component
      • Doll’s eyes (oculocephalic reflex) – Passive movement of the head to the right and left.  The normal intact brainstem response is for the eyes to remain fixated on a spot in space and for the eyes to move within the socket.  If the eyes remain “painted on” and don’t move with movement of the head, this indicates an absent brainstem response.
      • Cold calorics (oculovestibular reflex) – A stronger reflex response than the Doll’s eyes maneuver, cold water is injected into an ear.  A normal intact brainstem response in a comatose patient is for the eyes to deviate conjugately to the ipsilateral ear until the tympanic membrane temperature returns to normal at which point the eyes return to midline.  Injection of cold water into the opposite ear results in conjugate deviation of the eyes to the ipsilateral ear.  Failure of the eyes to deviate indicates an absent brainstem response.
  • Differential diagnosis of vertigo
    • Meniere’s disease
      • Episodes of vertigo, nausea, vomiting, diminished hearing, and tinnitus
      • Treatment: Restrict salt/caffeine/tobacco, use of diuretics, meclizine, benzodiazepines
    • Benign positional vertigo
      • Recurrent episodes of vertigo lasting ≤ 1 minute and may be associated with nausea and vomiting
      • Provoked by looking up while standing or sitting, lying down or getting up from bed, and rolling over in bed
      • Typically recur periodically for weeks to months without therapy
      • May have evidence of prior inner ear damage
      • Diagnosis: Dix-Hallpike maneuver
      • Treatment: Epley or Barany maneuvers
    • Labyrinthitis
      • Prolonged, severe episodes of vertigo accompanied by nausea, vomiting, and disequilibrium caused by inflammation or dysfunction of the vestibular apparatus

Cranial Nerve IX – Glossopharyngeal nerve

  • Evaluation: Afferent limb of the gag reflex.  CN X is the efferent limb that results in palatal elevation.

Cranial Nerve X – Vagus nerve

  • Evaluation: Efferent limb of the gag reflex that causes palatal elevation.  The afferent limb is CN IX.

Cranial Nerve XI – Accessory nerve

  • Innervates the sternocleidomastoid and trapezius muscles
  • Evaluation
    • Sternocleidomastoid – Turning the head to one side results from contraction of the contralateral sternocleidomastoid (Click here for Picture)
    • Trapezius – Shrugging the shoulder results from contraction of the ipsilateral trapezius

Cranial Nerve XII – Hypoglossal nerve

  • Evaluation: Have the patient stick out their tongue and move it side to side

“Bulbar weakness” - Symptoms such as dysphagia, dysarthria, diplopia, dyspnea which localize to the “bulb” or the brainstem

  • Differential diagnosis – This is not an exhaustive list, but 2 common conditions to consider include:
    • Myasthenia gravis
    • ALS

Horner’s syndrome

  • Injury to the sympathetic chain resulting in ptosis, meiosis, and anhydrosis
  • Differential diagnosis
    • Brainstem stroke
    • Tumor in lung apex (pancoast tumor)
    • Carotid dissection

CASE 1

70 year-old man with a history of hypertension and diabetes develops sudden onset of double vision and pain over the right temple.  Neurologic examination reveals mild ptosis of the right eye, failure of the right eye to adduct and impaired upgaze on the right.  The pupils are reactive. (Click on your choice to see the answers)

  1. A lesion in which cranial nerve would cause these exam findings?
    1. Cranial nerve II
    2. Cranial nerve III
    3. Cranial nerve IV
    4. Cranial nerve V
    5. Cranial nerve VI
    6. Cranial nerve VII

  2. What is the most likely etiology of the cranial nerve dysfunction?
    1. Aneurysm?
    2. Infarct of CN III?

CASE 2

45 year-old man with a 40 pack year smoking history presents with ptosis on the left, and the left pupil is found to be smaller than the right but is reactive.  The rest of his exam is otherwise normal.

  1. What is this syndrome called?
  2. What is the most likely cause in this patient?

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8. TEMPORAL ARTERITIS

Temporal arteritis or giant cell arteritis, is caused by an inflammatory response to medium and large arteries of the head and neck primarily and may extend into the carotids and aorta.  New-onset headache in any patient older than 50 years prompts consideration of this diagnosis, or if there has been a change in character from a previous headache pattern.

  • The pain is typically:
    • Unilateral, classically in the temporal region, but may be diffuse or bilateral
    • Frequently worse at night
    • Burning or jabbing sensation over the scalp and face
    • Provoked by combing hair, wearing of a hat or glasses, or resting the head on a pillow
  • Other symptoms
    • Vision loss
      • May occur suddenly and painlessly
      • May be transient (amaurosis fugax-type picture) or permanent.
      • Blindness eventually occurs in 20-50% of untreated patients
    • Jaw claudication when chewing or talking
    • Constitutional symptoms include anorexia and weight loss, fever and sweats, and malaise, fatigue, and depression.
  • Based on the 1990 American College of Rheumatology criteria for classification of giant cell arteritis, 3 of the following 5 items must be present:
    • Development of symptoms in patients older than 50 years
    • New onset of headache or localized head pain
    • Temporal artery tenderness to palpation
    • Decreased pulsations not related to arteriosclerosis of cervical arteries
    • Erythrocyte sedimentation rate (ESR) greater than 50 mm/h
  • Diagnosis
    • Labs
      • CBC
      • ESR
        • Most patients with temporal arteritis have an ESR > 80.
        • Approximately 10% of patients may have an ESR < 30.
      • CRP
        • May be elevated (>2.4)
        • CRP level may even be elevated in some patients with a normal ESR
    • Definitive diagnosis = temporal artery biopsy.
      • Involvement tends to be patchy or segmental so an adequate biopsy needs to be taken (20mm) and pathology needs to perform serial sections
      • Steroid therapy may affect results of biopsy, but inflammatory changes usually persist for 2-4 weeks after initiation of treatment
  • Treatment
    • High dose prednisone or IV methylprednisolone (Solumedrol)
    • Ophthalmology consult
    • Rheumatology or internal medicine consult to manage care

CASE

66 year-old woman with a new onset of headache which began a few weeks ago.  The pain is located over the left temporal head region, and the scalp is very tender to palpation.  She became frightened because yesterday while shopping, she temporarily lost vision in her left eye as if a curtain came over her vision.  She has been experiencing fever and night sweats. (Click on the questions to see the answers)

  1. What is the likely diagnosis?
  2. What tests should be ordered to confirm the diagnosis?
  3. How should this be treated?

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9. PERIPHERAL NEUROPATHY

Damage to the distal nerves in the hands and feet cause a sensation of numbness, pins/needles, burning, and/or electrical pain in the affected areas.  The typical peripheral neuropathy is a length-dependent process, so the feet (or “stockings”) become affected earlier than the hands (or “gloves”).  Not until the symptoms have progressed to above the knees do the hands typically become involved.  The patient, however, may have superimposed carpal tunnel syndrome, etc, which could give symptoms in the hands earlier, such as at the onset of the symptoms in the feet.  The typical peripheral neuropathy progresses over weeks to months in a gradually progressive manner.

Physical exam findings

  • Motor
    • Possibly diminished grip strength and/or foot drop if the motor nerves are affected. Toe flexion and extension are frequently weak.
  • Sensory
    • Diminished pinprick in a distal gradient especially in the feet
    • Possible diminished proprioception and vibration in a distal gradient especially in the feet
  • Reflexes
    • Normal to perhaps slightly diminished reflexes in the arms
    • Depressed reflexes at the knees
    • Absent ankle jerks

Diagnosis

  • Electromyogram and nerve conduction studies (EMG/NCS)

Differential diagnosis

  • Diabetes - the most common etiology in developed countries.
  • B12 deficiency
  • Hypothyroidism
  • Alcoholism
  • Drug induced
  • Heavy metal poisoning
  • Factoid: The most common worldwide cause of peripheral neuropathy is leprosy

Treatment

  • Neurontin (gabapentin), Lyrica (pregabalin), tricyclic antidepressants, and Cymbalta can help with the pain of neuropathy but do not improve the sensation (i.e. relieve the numbness).

Guillain-Barré Syndrome (GBS)

Rapidly progressive weakness with some sensory loss over the course several days or a week or two.  The course of the condition is much faster than that of typical peripheral neuropathy.  A mild respiratory or GI illness often precedes the symptoms by 1-3 weeks.

  • Symptoms of GBS
    • Classically described as an ascending paralysis, however this does not have to be the case
    • Respiratory involvement is the most worrisome symptom as death can occur from respiratory failure
    • Often describe an achiness in the muscles of the hips, thighs, and back
  • Neurological Exam
    • Reduced and then absent reflexes
    • At an early stage, the arm muscles may be less weak than the leg muscles
    • May have facial weakness
    • Perhaps diminished vibration more than pain/temperature, but weakness is much more prominent
    • Often have autonomic dysfunction
  • Diagnosis
    • EMG/NCS
    • Lumbar puncture
      • Look for albuminocytologic dissociation (high protein, which could possibly indicate some sort of infection, however the number of white cells is normal showing that there is no infection). The elevated protein is due to demyelination of the motor nerves.
  • Treatment
    • Plasma exchange or IVIg

CASE

62 year-old man presents with a couple of month history of numbness and tingling and pain which began in his toes and has progressed to mid-foot.  He describes a pins and needles sensation with some burning pain.  He has also been tripping on his feet somewhat, and his gait has become unsteady.

Neurologic Examination:  Normal strength throughout except mild weakness on ankle dorsiflexion and toe extension.  Diminished pinprick to the ankles with diminished but present vibration and proprioception at the toes.  Reflexes are normal in the arms, diminished at the knees, and absent at the ankles. Toes are mute.  The remainder of the neurologic examination is normal except decreased height on heel walk and unsteadiness on tandem gait. (Click on questions to see the answers)

  1. What is the diagnosis?

  2. What test would prove the diagnosis?
    1. Electromyography/nerve conduction (EMG/NCS) study
    2. MRI of the lumbar spine
    3. Muscle biopsy
    4. Nerve biopsy

  3. What labs would you order to determine the etiology?

  4. What treatment is available to improve the patient’s ability to sense his feet, improve his numbness?

  5. Which medications are helpful in treating the pain and discomfort?
    1. Neurontin (gabapentin)
    2. Lyrica (pregabalin)
    3. Tricyclic antidepressants like amitriptyline, nortriptyline
    4. Cymbalta (duloxetine)

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10. DEMENTIA

Dementia: Decline in cognition (memory, executive function (planning / organization), language, or orientation) that interferes with everyday function.

Alzheimer’s disease

  • Most common cause of dementia (60-80%)
  • Usually occurs in patients age 65 or older
  • Marked by early memory impairment and executive dysfunction
    • Examples of memory impairment
      • Forgetfulness (conversations; appointments; medicines; names)
      • Repetition of questions, statements
      • Misplacement of items
    • Examples of executive dysfunction
      • Managing household finances
      • Driving
      • Meal preparation
      • Operating appliances
  • Must have deficits in at least 2 of the following areas of cognition
    • Memory
    • Orientation
    • Judgment and Problem Solving
    • Community Affairs
    • Home and Hobbies
    • Personal Care
  • Gradual onset and progressive decline over months or years.  The average life expectancy after onset of symptoms is approximately 8 years, but progression may occur as short as 3 years or as long as 20 years.
  • Treatment
    • Acetylcholinesterase inhibitor such as donepezil (Aricept), rivastigmine (Exelon), or galantamine (Razadyne)
    • NMDA receptor antagonist such as memantine (Namenda)

Dementia with Lewy Bodies

  • Clinical diagnosis
    • Dementia
    • Two of the following
      • Fluctuations in cognition
      • Visual Hallucinations
      • Parkinsonism
    • Supportive features: falls, syncope, neuroleptic sensitivity, delusions, loss of consciousness

Frontotemporal Dementia (Pick’s disease = FTD syndrome with Pick bodies at autopsy)

  • Clinical features
    • Early behavioral and/or language deficits
    • Relative sparing of memory (Click here for picture 1 and picture 2)

Vascular Dementia (multi-infarct dementia) (Click here for Picture)

  • Focal neurologic signs
  • Stepwise progression
  • Gait difficulties, urinary incontinence, parkinsonian features
  • Subcortical dementia
    • Slowing of thought
    • Apathy

Normal Pressure Hydrocephalus

  • Clinical triad:
    • Wet (urinary incontinence)
      • Early on, urgency and frequency
      • Later,  true incontinence
    • Wobbly (gait disorder)
      • Bradykinetic
      • Broad-based
      • Shuffling
      • Apraxic, “magnetic” (like the feet are stuck to the floor)
      • Marked difficulty taking the first step (start hesitation) or turning
    • Wacky (dementia)
  • “Normal pressure” is actually a misnomer since there is probably intermittent transient elevations in intracranial pressure with the pressure returning to normal transiently after ventricular dilatation
  • Causes
    • Head injury
    • Subarachnoid hemorrhage
    • Meningitis
    • CNS tumor
  • Diagnosis
    • Imaging
      • Ventricular enlargement out of proportion to sulcal atrophy (Click here for Picture)
    • High-volume lumbar puncture (30-50cc of CSF is removed) with timed walk before and after to determine if the patient is a shunt candidate
      • Prior to LP, have the patient walk a set distance, time the walk, count the steps, and describe the gait in detail
      • Immediately following the LP, repeat the timed walk.  If the patient is a shunt candidate, there should be improvement in the gait
      • 3 hours after the LP, again repeat the timed walk.  There is usually mild worsening of the gait compared to immediately after the LP
      • Repeat the timed walk the next morning.  The gait usually returns to baseline by this time.
      • If there is improvement in the gait following a high-volume LP, consult neurosurgery for consideration of shunt placement

Creutzfeldt-Jakob (CJD)

  • Symptoms
    • Rapidly progressive form of dementia, usually with death within 1 year
    • Over the course of a few months, the patient becomes lost in their own home and has significant changes in memory and judgment
    • Develop myoclonus or involuntary muscle jerks
    • Visual disturbances
  • Diagnosis
    • MRI brain can show hyperintensities in the cortex and cortical ribboning in the basal ganglia
    • Definitive diagnosis is made at autopsy, showing spongiform changes in the cortex
    • EEG may show periodic complexes

Wernicke’s encephalopathy

An acute confusional state, ophthalmoplegia, and ataxia caused by thiamine deficiency.

 

CASE

The following are descriptions of paitents with dementia. Which type of dementia is best characterized by the following descriptions? (Click to see correct answer)

  1. 72 year-old woman with 2 year history of gradually progressive memory loss, repeating herself, mild disorientation to time.
    1. Alzheimer’s disease (AD)
    2. Creutzfeldt-Jakob (CJD)
    3. Dementia with Lewy bodies (DLB)
    4. Frontotemporal dementia (FTD)
    5. Normal pressure hydrocephalus (NPH)
    6. Vascular dementia (Multi-infarct dementia)

  2. 68 year-old woman with several month history of urinary incontinence, gait difficulties, and memory loss.
    1. Alzheimer’s disease (AD)
    2. Creutzfeldt-Jakob (CJD)
    3. Dementia with Lewy bodies (DLB)
    4. Frontotemporal dementia (FTD)
    5. Normal pressure hydrocephalus (NPH)
    6. Vascular dementia (Multi-infarct dementia)

  3. 55 year-old man with a 1 year history of disinhibition and word-finding difficulties
    1. Alzheimer’s disease (AD)
    2. Creutzfeldt-Jakob (CJD)
    3. Dementia with Lewy bodies (DLB)
    4. Frontotemporal dementia (FTD)
    5. Normal pressure hydrocephalus (NPH)
    6. Vascular dementia (Multi-infarct dementia)

  4. 49 year-old woman with a couple of month history of rapidly progressive memory loss, now getting lost in her own home, with associated movement disorder and periodic complexes on EEG
    1. Alzheimer’s disease (AD)
    2. Creutzfeldt-Jakob (CJD)
    3. Dementia with Lewy bodies (DLB)
    4. Frontotemporal dementia (FTD)
    5. Normal pressure hydrocephalus (NPH)
    6. Vascular dementia (Multi-infarct dementia)

  5. 83 year-old man with a history of diabetes and hypertension with a 2 year history of step-wise progression of memory loss
    1. Alzheimer’s disease (AD)
    2. Creutzfeldt-Jakob (CJD)
    3. Dementia with Lewy bodies (DLB)
    4. Frontotemporal dementia (FTD)
    5. Normal pressure hydrocephalus (NPH)
    6. Vascular dementia (Multi-infarct dementia)

  6. 69 year-old man with frequent falls, shuffling gait, memory loss, and visual hallucinations
    1. Alzheimer’s disease (AD)
    2. Creutzfeldt-Jakob (CJD)
    3. Dementia with Lewy bodies (DLB)
    4. Frontotemporal dementia (FTD)
    5. Normal pressure hydrocephalus (NPH)
    6. Vascular dementia (Multi-infarct dementia)

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