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Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

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Page 1: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Where’s the Lesion?

Neuroscience Core Lecture Series23 October 2002

David Roman Renner, MDDepartment of Neurology

Page 2: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Scott’s CC:

“My balance is off.” Multiple ER visits for fall-related trauma

“I’m losing the fine control of my fingers.” Loss of manual dexterity

“I’ve had pneumonia three times.” Dysphagia to liquids>solids

Page 3: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

All of Scott’s Complaints Sounded Neurologic in Origin

His lesion should lie somewhere in the

neuraxis.

Page 4: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Divisions of the Neuraxis

Cortical BrainSubcortical

BrainBrainstemCerebellumSpinal CordRootPeripheral

Nerve

Neuromuscular Junction

Muscle

Page 5: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Off the Top of my Head . . .

Imbalance = Cerebellum

Pneumonia = Brainstem (related dysphagia)

Loss of Dexterity = Peripheral Nerve

Page 6: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Neurologic Examination

Higher Cortical FunctionCranial NervesCerebellar Function MotorSensoryDeep Tendon ReflexesPathologic Reflexes

Page 7: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

The Neuro Exam Should Evaluate the Entire Neuraxis

Higher Cortical Function: cortexCranial Nerves: subcortex, brainstemCerebellar Function: cerebellumMotor: motor homonculous, subcortical

pyramidal tracts, BS, cord, radicle, PN, muscleSensory: ascending tracts, thalamus,

subcortical tracts, sensory hononculousDeep Tendon Reflexes: afferent PN, radicle,

cord, efferent PN, musclePathologic Reflexes:

Page 8: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Scott’s Exam Showed:

Higher Cortical Function: normalCranial Nerves: oropharyngeal dysarthriaCerebellar Function: hypotonia, assynergy,

dysmetria, staccato dysarthria, intention tremor, appendicular ataxia

Motor: hypotonia, normal strengthSensory: decreased vibration and temperatureDeep Tendon Reflexes: areflexiaPathologic Reflexes: plantar flexing

Page 9: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

So Where’s the Lesion?

Page 10: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Divisions of the Neuraxis

Cortical BrainSubcortical

BrainBrainstemCerebellumSpinal CordRootPeripheral

Nerve

Neuromuscular Junction

Muscle

Page 11: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Cortical Brain

Depends upon hemispheric dominance

Non-neurologists generalize: right: visual/spatial, perception and memory left: language and language dependent

memory

Look for aphasias, apraxias, and agnosias

Page 12: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Neurologic Examination when Cortical Brain is

Lesioned

Higher Cortical Function: aphasia, apraxia, agnosia

Cranial Nerves: normal Cerebellar Function: normal Motor: weakness if you hit the motor

homonculous Sensory: sensory abnormalities if you hit the

sensory homonculous Deep Tendon Reflexes: hemi-hyper-reflexia Pathologic Reflexes: possibly Babinski’s reflex or

frontal release signs

Page 13: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Divisions of the Neuraxis

Cortical BrainSubcortical

BrainBrainstemCerebellumSpinal CordRootPeripheral

Nerve

Neuromuscular Junction

Muscle

Page 14: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Subcortical Brain

Deep white radiating fibers produce equal involvement of face/arm/leg weakness sensory abnormalities

Visual radiating fibers are interrupted: deep parietal: pie on the floor deep temporal: pie in the sky

Page 15: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Neurologic Examination when Subcortical Brain is

Lesioned

Higher Cortical Function: normal Cranial Nerves: visual field cuts Cerebellar Function: usually normal Motor: weakness in face=arm=leg, UMN Sensory: sensory abnormalities in face=arm=leg Deep Tendon Reflexes: hemi-hyper-reflexia Pathologic Reflexes: Babinski’s reflex and

possibly frontal release signs

Page 16: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Divisions of the Neuraxis

Cortical BrainSubcortical

BrainBrainstemCerebellumSpinal CordRootPeripheral

Nerve

Neuromuscular Junction

Muscle

Page 17: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Brainstem

The Brainstem is basically spinal cord with embedded cranial nerves, producing the following abnormalities cranial nerve abnormalities classic spinal cord complaints

bowel and bladder problems

long tract signs: (bilateral and crossed)corticospinal (pyramidal): motorspinothalamic: pain/temp to the thalamusdorsal columns: prioprioception/vibration to

thal.

Page 18: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Neurologic Examination when Brainstem is Lesioned

Higher Cortical Function: normal Cranial Nerves:

III, IV, VI: diplopia V: decreased facial sensation VII: drooping VIII: deaf and dizzy IX, X, XII: dysarthria and dysphagia XI: decreased strength in neck and shoulders

Cerebellar Function: normal Motor: hemi-paresis, UMN Sensory: hemi-dysesthesias Deep Tendon Reflexes: hemi-hyper-reflexia Pathologic Reflexes: Babinski’s reflex

Page 19: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Divisions of the Neuraxis

Cortical BrainSubcortical

BrainBrainstemCerebellumSpinal CordRootPeripheral

Nerve

Neuromuscular Junction

Muscle

Page 20: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Cerebellar Function

Some people believe that one can not test specifically for cerebellar abnormalities no one test on examination reliably evaluates the cerebellum

H: hypotoniaA: assynergy of (ant)agonist musclesN: nystagmusD: dysmetria, dysarthriaS: stance and gaitT: tremor

Page 21: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Neurologic Examination when the Cerebellum is

Lesioned

Higher Cortical Function: normal Cranial Nerves: normal Cerebellar Function:

nystagmus staccato dysarthria (abnormality of prosody)

Motor: hemi-hypotonia intention > positional tremor axial instability with dysmetria

Sensory: normal Deep Tendon Reflexes: normal Pathologic Reflexes: none

Page 22: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Divisions of the Neuraxis

Cortical BrainSubcortical

BrainBrainstemCerebellumSpinal CordRootPeripheral

Nerve

Neuromuscular Junction

Muscle

Page 23: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Spinal Cord

Sensory level (horizontal)Weakness below the lesion

(paraparesis)UMN signs below the lesion Bowel and bladder incontinence

Page 24: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Neurologic Examination when the Spinal Cord is

Lesioned

Higher Cortical Function: normalCranial Nerves: normalCerebellar Function: normalMotor: weakness below the lesionSensory: horizontal levelDeep Tendon Reflexes: hyper-reflexia

below the lesionPathologic Reflexes: Babinski’s reflex

Page 25: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Divisions of the Neuraxis

Cortical BrainSubcortical

BrainBrainstemCerebellumSpinal CordRootPeripheral

Nerve

Neuromuscular Junction

Muscle

Page 26: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Root/Radiculopathy

Pain is the hallmark of a radiculopathySensory abnormalities in a dermatome

provocative maneuvres exacerbate the pain

Weakness in a myotome (assymetric)LMN findings

Page 27: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Neurologic Examination when a Root is Lesioned

Higher Cortical Function: normalCranial Nerves: normalCerebellar Function: normalMotor: assymetric weakness in a

myotomeSensory: pain and dysesthesia confined to

a dermatomeDeep Tendon Reflexes: hypo- to a-reflexia

if the root carries a reflexPathologic Reflexes: none

Page 28: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Divisions of the Neuraxis

Cortical BrainSubcortical

BrainBrainstemCerebellumSpinal CordRootPeripheral

Nerve

Neuromuscular Junction

Muscle

Page 29: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Peripheral Nerve(presuming nonfocality)

Weakness: distal predominantSensory Dysesthesias: distal

predominant

Page 30: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Neurologic Examination with Diffuse PN Lesioning

Higher Cortical Function: normalCranial Nerves: normalCerebellar Function: normalMotor: weakness is distal predominant Sensory: dysesthesias are distal

predominantDeep Tendon Reflexes: loss of distal

reflexesPathologic Reflexes: mute responses to

plantar stimulation

Page 31: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Divisions of the Neuraxis

Cortical BrainSubcortical

BrainBrainstemCerebellumSpinal CordRootPeripheral

Nerve

Neuromuscular Junction

Muscle

Page 32: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Neuromuscular Junction

Fatiguability is the hallmarkWeakness: proximal and symmetric

exacerbated with use, recovers with rest

often affects facial muscles (ptosis, dysconjugate gaze, slack jaw)

Sensation: preserved

Page 33: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Neurologic Examination in Disorders of the NMJ

Higher Cortical Function: normal Cranial Nerves: fatiguabile ptosis, dysconjugate

gaze, slack jaw Cerebellar Function: normal Motor: fatiguable proximal weakness in both UE’s

and LE’s Sensory: normal Deep Tendon Reflexes: normal Pathologic Reflexes: none

Page 34: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Divisions of the Neuraxis

Cortical BrainSubcortical

BrainBrainstemCerebellumSpinal CordRootPeripheral

Nerve

Neuromuscular Junction

Muscle

Page 35: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Muscle

Weakness of proximal arm and leg muscles symmetric

Sensation is normal though patients complain of cramping

and aching

Page 36: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Neurologic Examination in Disorders of Muscle

Higher Cortical Function: normal Cranial Nerves: ptosis, dysconjugate gaze,

dysphagia, dysphonia, (dysarthria) Cerebellar Function: normal Motor: proximal weakness in both UE’s and LE’s,

atrophy and fasiculations, hypotonia Sensory: normal Deep Tendon Reflexes: preserved until late in the

disease Pathologic Reflexes: none

Page 37: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

Scott’s Lesion Localizes to:

Almost exclusively the Cerebellum, though to a minor degree the BS and PN are involved.

Isolated heritable cerebellar dysfunction is rare, so we would expect to see other parts of the CNS involved.

Page 38: Where’s the Lesion? Neuroscience Core Lecture Series 23 October 2002 David Roman Renner, MD Department of Neurology

SpinoCerebellar Ataxia (SCA4)

Prior to Scott’s diagnosis, his cousin was the proband for this entity. Scott has a 38-family member, 5 generation pedigree of this

disorder

His family entered into a study, and his family led to the classification of SCA4:ataxia with axonal sensory neuropathy


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