CLINICAL CASE 554 04/20/10 Unconsciousness

History Medical history

• 61 year old security guard • Alcohol addiction
• Found unresponsive on the ground • Untreated hypertension for (at least) 28 years
• Vomited • 2000mg ASA/day – blood thinner – increases
• Bruise on the side of the head severity of bleeding
Physical examination
• High blood pressure, fast, irregular heartbeat
• Cheyne-Stokes breathing: indicates a lesion in the cerebral hemispheres
• Dupuytren’s contractures in both hands: fixed flexion contracture of fingers on the hand that cannot be extended
• Spider angiomas
• Mental status: unconscious
• Painful stimulus:
o Right arm flexion: decorticate posturing – lesion above the left red
nucleus
o Left arm and both legs extended: decerebrate posturing – lesion at
or below the right red nucleus
o Bilateral extensor plantar response: upper motor neuron lesion
• Cranial nerve functions:
o Right pupil wider than left pupil: right CN III lesion – unopposed
sympathetic innervation
o Right pupil doesn’t react to light: right CN III lesion
o Both eyes deviated toward the right side: right cortical or left pontine lesion

Lesion
• Vascular
• Central:
o Right cortex
o Right and left midbrain
• Two possibilities:
o Right intracerebral hemorrhate (hypertensive)
o Epidural hematoma (traumatic) seizure in alcohol-intoxication or withdrawal
o Both increase intracranial pressure and can cause uncal herniation
• Uncal herniation: puts pressure on the brainstem, specifically the midbrain.
o Pressure on nearby CN III – affects parasympathetic innervation
o Disrupted brainstem leads to decorticate posturing
CLINICAL CASE 555 04/21/10 Paresthesia

History
• 24 year old female college student
• Right-sided numbness worsening over the last three weeks
• Three weeks of left facial tingling last year
• Right hand/forearm numbness two years ago
• Blurred vision five years ago
Examination
• Right optic disc appears pale: papilledema, demyelination
• Right eye horizontal nystagmus
• Incomplete left eye adduction: left internuclear opthalmoplegia (MLF syndrome)
o Convergence would be intact because it uses a different route than the PPRF for adduction
• Diminished proprioception and vibration of the right arm and leg: right dorsal
columns and left medial leminiscus damaged
• Increased muscle tone in both legs: upper motor
neuron lesion
• Deep tendon reflexes: 3+ bilaterally: upper motor
neuron lesion
• Plantar extensor response bilaterally (Babinski
sign positive): upper motor neuron lesion
• Normal muscle bulk and power
• Right side ataxia: cerebellar damage – ataxia is ipsilateral to the side of the cerebellar lesion
• Unstable when standing with feet together and worse with eyes closed.
o Three balance systems: visual, vestibular, proprioceptive
Lesion
• Multiple lesions in space and time
• Inflammatory
• Most likely diagnosis: multiple sclerosis: central demyelination syndrome more common in women.
• Tests for confirmation:
o T1 MRI with contrast: shows paraventricular ovoid plaques.
o Lumbar puncture: test for oligoclonal bands, myelin basic protein, igG synthetic rate, WBCs,
etc.
o Evoked optic potentials: track optic signals as they travel from the eye to find any latencies that
would coincide with optic neuritis (usually t he first symptom
of multiple sclerosis).
• Treatment
o Acute attack: cortisone
o Chronic: immunosuppressants (Azathioprine), Interfero n-1b,
interferon-1a, gatiramer acetate (Copaxone)
CLINICAL CASE 556 04/22/10 Paralyzed legs

History
• 26 year old, female polytoxicomaniac (drug user) • In wheelchair
• In methadone program • Since morning not able to walk
• Since 2 days progressive weakness and numbness in • Since morning not able to comb hair – weakness in
both legs both arms
Medical History
• Gastroenteritis (viral) four weeks previously with diarrhea and gastrointestinal cramping
Examination
• Pulse 88/min
• Blood pressure 130/80
• Respiration 20/min: hyperventilation
• Nail beds and buccal mucosa cyanotic: oxygenation of blood not sufficient
to perfuse tissue
• Bilateral facial weakness: damage to nerves (e.g. a patient with right
central/cortical facial paralysis is able to raise both eyebrows)
• Bilateral lower limb paralysis
• Reflexes: 1+ bilaterally in upper limbs
(decreased), absent in lower limbs, no
Babinski sign bilaterally: upper motor
neuron lesion
• Unable to raise from wheelchair without
help: corticospinal tract
• Could not clench fists bilaterally:
corticospinal tract
• Could not raise arms higher than shoulder: corticospinal tract
• Bilateral decreased pain and temperature perception in both lower limbs: spinothalamic tract
• Bilateral decreased vibratory perception in both lower limbs: dorsal column-medial leminiscus pathway
• Absent joint position perception: dorsal column-medial leminiscus
pathway
• Nerve conduction study:
o Left leg: 10 m/s Right leg: 12 m/s
o Left arm: 39 m/s Right arm: 33 m/s
o Normal: 55-60 m/s
• CSF:
Measured Normal
3
WBC: 2/mm 4/mm3
3
RBC: 0/mm 0/mm3
Glucose: 63 mm/dl 75 mm/dl
Protein : 84 mg/dl 23 mg/dl
Lesion
• Peripheral nerves affected
• Diagnosis: Guillain-Barre Syndrome – acute inflammatory demyelinating
polyneuropathy
CLINICAL CASE 557 04/23/10 Dizziness

History

• 42 year old housewife

• Sudden severe dizziness – kitchen was spinning
• Feeling falling to the left
• Unable to stand
• Nausea, vomited twice

Medical history

• Milder and briefer similar episode the day before

• 3 similar milder episodes over the last 6 months
• Hearing deteriorated on the left side since 6 months
• Constant ringing sound, louder in the left

Examination

• Dizzy, nauseous
• Prefers to lie on the right side: vestibular system problem
• Pale, diaphoretic (sweaty)
• BP = 130/85 mmHg, P = 56/min, respiration 13/min
• Acute vertigo
• Sense of fullness in the left ear
• Unable to detect sound of fingers being rubbed together next to the
left external auditory meatus: unilateral hearing loss indicates peripheral lesion in the cochlea or CN VII
• Horizontal nystagmus (slow phase to the left, quick phase to the right): lesion in the left vestibular organ (labyrinth)

Lesion

• Left, peripheral
• Meniere’s disease: labyrinth and part of the cochlea are swollen by an unknown cause, causing excess fluid in the
inner ear.
o Treatment:
! Intratympanic treatment (chemical labyrinthotomy), gentamicin injection – affects hearing
! Endolymphatic shunt or decompression procedure – preserves hearing
! Selective vestibular neurectomy (vestibular nerve is cut)
! Eighth nerve section – hearing not preserved
! Labrynthectomy – hearing not preserved
CLINICAL CASE 558 04/27/10 Falling to the right side

History
• 56 year old male, civil engineer
• Felt suddenly unstable and dizzy
• Almost fell in excavation
• Left hand and fingers numb
• Persistent hiccups
• Nauseous and vomited
Examination
• Poorly controlled type II diabetes
• Blood pressure 150/95 mmHg
• Pulse 96/min
• Respiration 18/min
• Cranial nerve functions:
o Pupils (R/L) 2mm/4mm, reacting to light 1.5mm/2.5mm: Horner’s syndrome
o Right partial ptosis: Horner’s syndrome
o Decreased sensation to pain and temperature over the right face: ipsilateral
lesion CN V
o Singultus: reticular formation
o Dysarthria
o Dysphagia: CN IX, X
o Palate did not elevate on the right: lesion to the left corticobulbar fibers, right CN X, or right nucleus
ambiguus
• Sensory-motor:
o Normal strength, tone, and bulk
o Muscle stretch reflexes normal in arms and legs
o Flexor plantar response
o Decreased pain and temperature perception over left arm, trunk, and leg: right ALS
o Tandem gait not possible, veering to the right: right vestibular nucleus
Lesion
• Infarction of the dorsolateral medulla
oblongata – occlusion of the right vertebral
artery
• Right Wallenberg’s syndrome (aka
dorsolateral medulla oblongata syndrome) –
occlusion of the posterior inferior cerebellar
artery or right vertebral artery
o Crossed somatosensory symptoms
o Horner’s syndrome
CLINICAL CASE 559 04/28/10 Memory loss

History
• 62 year old male, unknown occupation, alcoholic
• Hit his head when he fell several weeks prior resulting in 15 minute loss of consciousness
• Smelled ethanol on his breath, drinks 1.5 pints of rum a day
Examination
• Nystagmus in all directions of gaze: lesion in the vestibular organ, nerve or nucleus, or lesion in the cerebellum
• Muscle strength 5/5 bilaterally in all extremities (full): intact corticospinal tract
• Dysdiadokinesia (inability to perform rapid, alternating movements): cerebellar lesion
• Intention tremor: cerebellar lesion
• Decreased vibration and proprioception in the feet: caused by alcohol – alcoholic neuropathy
• Intact tendon reflexes except loss of ankle jerks: problem with muscle spindles – alcoholic polyneuropathy
• Disorientation to time, place, and person: cortex/subcortex memory circuits
• Unstable, wide-based and magnetic gait:
o Acute: cerebellar ethanol intoxication
o Chronic: cerebellar degeneration (atrophy)
o Chronic: peripheral neuropathy
Lesion
• Toxic/metabolic/nutritional – both brain and peripheral nerves affected
• Werrnicke’s Encephalopathy
o Inability to form new memories
o Loss of memory (can be severe)
o Loss of muscle coordination (ataxia): unsteady, uncoordinated walking
o Making up stories (confabulation)
o Seeing or hearing things that aren’t really there (hallucinations)
o Vision changes: abnormal eye movements, double vision, eyelid drooping
*not all symptoms have to be present
o Caused by lesions in the:
! Mammillary bodies
! Medial thalamic nuclei
! Periaqueductal brainstem nuclei
! Periventricular brainstem nuclei
! Cerebellar vermis
• Alcoholic peripheral neuropathy:
o Sensory and motor losses, both symmetric
o Starts with sensory neuropathy from distal extremity (foot, leg)
o Later motor loss starting a lower leg
o Nerve conduction velocity in most cases normal
o Vitamin B1 (thiamine) deficiency caused by malnutrition
• Treatment: thiamine – initially IV and then orally
• Korsakoff syndrome: anterograde and retrograde amnesia with confabulation
CLINICAL CASE 560 04/29/10 Sensory loss – upper extremity

History Medical history

• 46-year-old woman • Car accident at the age of 16
• Repeatedly burning her left hand • C3 and C4 fracture of vertebrae
• Could not feel pain in left hand • Contusion of spinal cord and quadriplegia
• Has felt pain and numbness in shoulders and arm • Recovered fully within one year
(left > right)
• Increased difficulties in walking during last month
Examination
• Medical and surgical history unremarkable
• Vital signs stable
• Third degree burns left hand
• Slow and shuffling gait
• Decreased pin-prick left arm (shoulder down): C6 lesion
• Decreased pin-prick right shoulder
• Non pin-prick perception left hand
• Reduced temperature perception left arm: ALS
• No temperature perception left hand: ALS
• Proprioception and vibration present in all extremities: dorsal column-medial leminiscus
• Diminished muscle bulk bilaterally in interosseus, thenar, hypothenar muscles: lower motor neuron syndrome
• Diminished bulk in left forearm: lower motor neuron syndrome
• Decreased muscle strength in upper extremities R/L 3/5: C5-T1 lesion
• Deep tendon reflexes (R/L) biceps 2+/1+, triceps 1+/1+, knee 4+/4+: upper body LMN, lower body UMN
Lesion
• Left and right central lesion: traumatic/degenerative
• Syringomyelia (syrinx of the spinal cord):
o Can have upper and lower motor neuron findings
o Affects a few levels of the spinal cord (
“segmental disease”)
o Lower motor neuron findings helps pinpoint what
spinal levels affected
o ALS often affected first
o Fewer sensory than motor levels (Lissauer’s tract)
• Additional testing: MRI-Spine
• Treatment: resection and
drainage of syrinx
CLINICAL CASE 561 04/30/10 Suicide attempt

History
• 32 year-old male
• Husband, no occupation known
• 2 year history of emotional instability with periods of lethargy and depression
• Occasional jerking movements of head, limbs, and trunk
• Unsteady gait
• Change in social behavior over past few months
Family History
• Grandfather died at an early age (50) – abnormal movement in arms and legs
Examination
• Oriented X3 • Abnormal movements in face, neck, trunk, and legs:
• 2/3 objects at 5 minutes chorea
• Good calculation skills • Normal strength
• Reduced visual-spacial skills • Very brisk reflexes (4+): cerebral control of reflexes
• Facial grimacing with eyebrow movements work well (upper motor neuron lesion)
• CN II –CN XII intact • Flexor plantar reflex
• Saccadic eye movements moderately slowed • Positive palmomental, snout, glabellar reflexes
• Unsteady tandem gait
Lesion
• Central , left and right
• Basal ganglia affected
• Genetic/hereditary
• Huntington’s disease: striatum is dying off
o The indirect pathway facilitates movement by inhibiting
striatal cells
o Symptoms:
! Mental disorder occurs prior to cognitive
dysfunction
! Depression in more than half
! Psychosis is common in advanced disease
! Diminished work performance
! Abnormality of movement first in hands/face
! Increased blinking and tongue protrusion
! No motor weakness
o Earlier onset in successive generations (anticipation) Procedures:
! MRI: Brain and cerebellar atrophy, ventricles and sulci widen
! DNA testing: autosomal dominant, more than 40 CGA trinucleotide repeats on chromosome 4q
o Mechanism: excess huntingtin aggregates in neurons of striatum and cortex (toxic to neurons)
! Leads to loss of D2 receptors and increased excitability of the motor cortex
o Neuronal death in striatum, cerebral cortex, hippocampus, cerebellum (Purkinje cells)
o Treatment: neuroleptics (block dopamine), tetrabenazine (depletes dopamine), amantadine (anticholinergic)
CLINICAL CASE 562 05/03/10 Gait disturbance and tremor

History
• 68 year old male, retired salesman
• Slowness/difficulty using right arm began three years ago
• Tremor in right arm and leg 2.5 years ago
Examination
• “Mask facies” – Low D1 Direct Pathway Low D2 Indirect pathway
expressionless/emotionless:
interpreted as a sad demeanor
• Hypophonic voice (“low talker”) Decreased Neostriatum output Increased Neostriatum output
• 4-5Hz tremor noted in right arm GABA GABA
and leg
Increased Gm output Decreased GPL output
• Cog-wheel rigidity in all limbs,
right > left GABA GABA
• Low finger-nose, heel-shin Decreased VA/VL output Increased Sth output
• Proximal weakness in thighs
Glutamate
(unable to rise from chair)
• Slow and shuffling gait with small Decreased cortical output Increased GPm output
strides GABA
• Lack of right arm swing
• Uses small steps to turn Decreased movement Decreased VA/VL output
• Unable to rise from chair without
assistance
Decreased cortical output
• Difficulty initiating movement
(akinesia)
• Postural instability Decreased movement

Lesion
• Parkinson’s disease: chronic, progressive disorder resulting from dopaminergic cell loss in the substantia nigra pars
compacta .
o Cardinal signs:
! Resting tremor
! Akinesia Parkinson’s disease Normal Midbrain
! Rigidity
! Loss of postural reflexes
• Degenerative, central, left and right lesion in the midbrain
• Function of the direct pathway: dopamine imput facilitates
movement by stimulating cells in the thalamus
• Additional tests: PET scan – reveals decreased dopamine activity in the basal ganglia
of the brain
• Treatment:
o Drugs: Dopamine agonists, L-Dopa, COMT inhibitors, MAO-B inhibitors
o Surgery:
! Deep brain stimulation (STN, GPi ) and pallidotomy of GPi
! Gene therapy, stem cell implantations
CLINICAL CASE 563 05/04/10 Paresthesia and neck pain

History
• 21 year old male, soccer player
• Left neck pain
• Left arm weakness
• Left neck and arm pain increasing since three weeks
• “Shooting” pain that radiates down the radial aspect of left forearm: C6, radial
• “Pins and needles” in the left thumb and index finger: radial nerve
• Pain is initiated to certain movements of head
• Pain gets worse when coughing or sneezing
• Cannot run anymore
• Walking less coordinated
• Urinary urgency: stretch receptors travel via ALS to brain
Examination
• Limited range of motion of neck
• Diminished strength in left elbow flexors: C5, C6
• Left brachioradialis reflex absent: C5, C6
• Left biceps reflex absent: C5, C6
• Legs slightly spastic
• Plantar reflex – extensor bilaterally
• Hyperreflexia in both legs: UMN (lesion of corticospinal
fibers)
• Tandem gait difficulty
• Left neck pain: localizing for the lesion – C6
• Left arm pain, radiating down lateral aspect of forearm into thumb
• Decreased pinprick sensation – left forearm and left thumb and index
finger: C6
• Tandem gait abnormal, casual walking normal:
• Coughing aggravates pain: increased pressure
Lesion
• Central, peripheral, left, sensory and motor
• Motor landmark: flexion of the elbow/brachioradialis muscle: C5, C6
• Subacute cervical radiculomyelopathy C5/C6:
o Tumor, abscess or other infectious mass
o Demyelination or other inflammatory lesion
o Fracture/dislocation
o Infarction
o Spondylosis
o Disc herniation (left lateral and medial herniation)
CLINICAL CASE 564 05/05/10 Weak neck muscles and choking
History
• 68 year old female
• Difficulties holding her head up
• Difficulties swallowing (dysphagia)
• Recent choking n food
• Difficulties walking
• Weakness in both hands (not able to knit)
• Dysarthria
Examination
• Vision full, pupils reactive to light; CN II
• Extraocular eye movements full: CN III, IV, VI
• Normal facial sensation CN V sensory
• Weakness of jaw opening, dysarthria: CN V motor
• Facial weakness: CN VII
• Hearing and balance normal: CN VIII
• Weakness of soft palate, increased gag reflex, dysarthria: CN IX, X
• Atrophy and fasciculations of tongue muscles, difficulties in protruding tongue (bilaterally): CN XII
• Generalized weakness of neck, arm and leg muscles, proximal and distal, arms weaker than legs: lower motor neuron
syndrome
• Atrophy and fasciculations in some muscles of arms and legs: lower motor neuron syndrome
! Analysis of symptoms:
• Pure motor
• Atrophy
• Fasciculations
• Progressive course
! Site of lesion: cell body of lower motor neuron
• Brisk jaw reflex
• Increased gag reflex
• Increased reflexes in upper and lower extremities: upper motor neuron syndrome
• Clonus in ankles: upper motor neuron syndrome
• Bilateral extensor plantar response: upper motor neuron syndrome
Lesion
• Central, bilateral
• In the pons, medulla, and spinal cord
• Signs of denervation:
o Fibrillations
o Giant units
o Normal conduction velocities
• Progressive illness (degenerative), pure motor, evidence for lower and
upper motor nerve syndromes
• Amytrophic Lateral Sclerosis: lower motor neuron lesion of the soma resulting in muscle weakness and difficulty
speaking and swallowing – upper motor
o Progressive loss of muscle strength
o Aspiration (recurrent pneumonia)
o Respiratory failure leading to death
CLINICAL CASE 565 05/06/10 The unsteady calculus professor

History
• Headache, sleepiness for one month
• Onset after falling from bed
• Recent left leg weakness
Examination
• Alert, oriented
• Short term memory: 2/3 objects
• Difficulty with serial 7’s (subtracting 7 from 100, then 93, etc.)
• Normal pupils and eye movements: CN II, III, IV, VI ok
• Left homonymous hemianopia: lesion in the right occipital lobe or right optic tract

• Flattened left nasolabial fold: right corticofacial fiber damage caused by a right cerebral infarction

• Normal gag reflex: CN IX, X ok

• Strength normal in trapezius and sternocleidomastoid muscles: CN XI ok
• Tongue protruded in midline: CN XII ok
• Left hemiparesis, pronator drift: right cerebral cortex lesion
Lesion
• Central lesion in the right cerebral hemisphere – traumatic
• Differential diagnosis:
o Normal pressure hydrocephalus: verify with spinal tap
o Binswanger’s disease: occlusion of small blood vessels in the corona radiate
o Meningeoma
o Subdural hematoma
! Tearing of bridging veins, crescent shaped CT