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Where

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... (ptosis, dysconjugate gaze, slack jaw) Sensation: preserved Neurologic Examination in Disorders of the NMJ Higher Cortical Function: normal Cranial Nerves: ... – PowerPoint PPT presentation

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Title: Where


1
Wheres the Lesion?
  • Neuroscience Core Lecture Series
  • 23 October 2002
  • David Roman Renner, MD
  • Department of Neurology

2
Scotts CC
  • My balance is off.
  • Multiple ER visits for fall-related trauma
  • Im losing the fine control of my fingers.
  • Loss of manual dexterity
  • Ive had pneumonia three times.
  • Dysphagia to liquidsgtsolids

3
All of Scotts Complaints Sounded Neurologic in
Origin
  • His lesion should lie somewhere in the neuraxis.

4
Divisions of the Neuraxis
  • Cortical Brain
  • Subcortical Brain
  • Brainstem
  • Cerebellum
  • Spinal Cord
  • Root
  • Peripheral Nerve
  • Neuromuscular Junction
  • Muscle

5
Off the Top of my Head . . .
  • Imbalance Cerebellum
  • Pneumonia Brainstem (related dysphagia)
  • Loss of Dexterity Peripheral Nerve

6
Neurologic Examination
  • Higher Cortical Function
  • Cranial Nerves
  • Cerebellar Function
  • Motor
  • Sensory
  • Deep Tendon Reflexes
  • Pathologic Reflexes

7
The Neuro Exam Should Evaluate the Entire
Neuraxis
  • Higher Cortical Function cortex
  • Cranial Nerves subcortex, brainstem
  • Cerebellar Function cerebellum
  • Motor motor homonculous, subcortical pyramidal
    tracts, BS, cord, radicle, PN, muscle
  • Sensory ascending tracts, thalamus, subcortical
    tracts, sensory hononculous
  • Deep Tendon Reflexes afferent PN, radicle, cord,
    efferent PN, muscle
  • Pathologic Reflexes

8
Scotts Exam Showed
  • Higher Cortical Function normal
  • Cranial Nerves oropharyngeal dysarthria
  • Cerebellar Function hypotonia, assynergy,
    dysmetria, staccato dysarthria, intention tremor,
    appendicular ataxia
  • Motor hypotonia, normal strength
  • Sensory decreased vibration and temperature
  • Deep Tendon Reflexes areflexia
  • Pathologic Reflexes plantar flexing

9
So Wheres the Lesion?
10
Divisions of the Neuraxis
  • Cortical Brain
  • Subcortical Brain
  • Brainstem
  • Cerebellum
  • Spinal Cord
  • Root
  • Peripheral Nerve
  • Neuromuscular Junction
  • Muscle

11
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

12
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 Babinskis reflex
    or frontal release signs

13
Divisions of the Neuraxis
  • Cortical Brain
  • Subcortical Brain
  • Brainstem
  • Cerebellum
  • Spinal Cord
  • Root
  • Peripheral Nerve
  • Neuromuscular Junction
  • Muscle

14
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

15
Neurologic Examination when Subcortical Brain is
Lesioned
  • Higher Cortical Function normal
  • Cranial Nerves visual field cuts
  • Cerebellar Function usually normal
  • Motor weakness in facearmleg, UMN
  • Sensory sensory abnormalities in facearmleg
  • Deep Tendon Reflexes hemi-hyper-reflexia
  • Pathologic Reflexes Babinskis reflex and
    possibly frontal release signs

16
Divisions of the Neuraxis
  • Cortical Brain
  • Subcortical Brain
  • Brainstem
  • Cerebellum
  • Spinal Cord
  • Root
  • Peripheral Nerve
  • Neuromuscular Junction
  • Muscle

17
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) motor
  • spinothalamic pain/temp to the thalamus
  • dorsal columns prioprioception/vibration to
    thal.

18
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 Babinskis reflex

19
Divisions of the Neuraxis
  • Cortical Brain
  • Subcortical Brain
  • Brainstem
  • Cerebellum
  • Spinal Cord
  • Root
  • Peripheral Nerve
  • Neuromuscular Junction
  • Muscle

20
Cerebellar Function
  • Some people believe that one can not test
    specifically for cerebellar abnormalities
  • no one test on examination reliably evaluates the
    cerebellum
  • H hypotonia
  • A assynergy of (ant)agonist muscles
  • N nystagmus
  • D dysmetria, dysarthria
  • S stance and gait
  • T tremor

21
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 gt positional tremor
  • axial instability with dysmetria
  • Sensory normal
  • Deep Tendon Reflexes normal
  • Pathologic Reflexes none

22
Divisions of the Neuraxis
  • Cortical Brain
  • Subcortical Brain
  • Brainstem
  • Cerebellum
  • Spinal Cord
  • Root
  • Peripheral Nerve
  • Neuromuscular Junction
  • Muscle

23
Spinal Cord
  • Sensory level (horizontal)
  • Weakness below the lesion (paraparesis)
  • UMN signs below the lesion
  • Bowel and bladder incontinence

24
Neurologic Examination when the Spinal Cord is
Lesioned
  • Higher Cortical Function normal
  • Cranial Nerves normal
  • Cerebellar Function normal
  • Motor weakness below the lesion
  • Sensory horizontal level
  • Deep Tendon Reflexes hyper-reflexia below the
    lesion
  • Pathologic Reflexes Babinskis reflex

25
Divisions of the Neuraxis
  • Cortical Brain
  • Subcortical Brain
  • Brainstem
  • Cerebellum
  • Spinal Cord
  • Root
  • Peripheral Nerve
  • Neuromuscular Junction
  • Muscle

26
Root/Radiculopathy
  • Pain is the hallmark of a radiculopathy
  • Sensory abnormalities in a dermatome
  • provocative maneuvres exacerbate the pain
  • Weakness in a myotome (assymetric)
  • LMN findings

27
Neurologic Examination when a Root is Lesioned
  • Higher Cortical Function normal
  • Cranial Nerves normal
  • Cerebellar Function normal
  • Motor assymetric weakness in a myotome
  • Sensory pain and dysesthesia confined to a
    dermatome
  • Deep Tendon Reflexes hypo- to a-reflexia if the
    root carries a reflex
  • Pathologic Reflexes none

28
Divisions of the Neuraxis
  • Cortical Brain
  • Subcortical Brain
  • Brainstem
  • Cerebellum
  • Spinal Cord
  • Root
  • Peripheral Nerve
  • Neuromuscular Junction
  • Muscle

29
Peripheral Nerve(presuming nonfocality)
  • Weakness distal predominant
  • Sensory Dysesthesias distal predominant

30
Neurologic Examination with Diffuse PN Lesioning
  • Higher Cortical Function normal
  • Cranial Nerves normal
  • Cerebellar Function normal
  • Motor weakness is distal predominant
  • Sensory dysesthesias are distal predominant
  • Deep Tendon Reflexes loss of distal reflexes
  • Pathologic Reflexes mute responses to plantar
    stimulation

31
Divisions of the Neuraxis
  • Cortical Brain
  • Subcortical Brain
  • Brainstem
  • Cerebellum
  • Spinal Cord
  • Root
  • Peripheral Nerve
  • Neuromuscular Junction
  • Muscle

32
Neuromuscular Junction
  • Fatiguability is the hallmark
  • Weakness proximal and symmetric
  • exacerbated with use, recovers with rest
  • often affects facial muscles (ptosis,
    dysconjugate gaze, slack jaw)
  • Sensation preserved

33
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 UEs
    and LEs
  • Sensory normal
  • Deep Tendon Reflexes normal
  • Pathologic Reflexes none

34
Divisions of the Neuraxis
  • Cortical Brain
  • Subcortical Brain
  • Brainstem
  • Cerebellum
  • Spinal Cord
  • Root
  • Peripheral Nerve
  • Neuromuscular Junction
  • Muscle

35
Muscle
  • Weakness of proximal arm and leg muscles
  • symmetric
  • Sensation is normal
  • though patients complain of cramping and aching

36
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 UEs and LEs,
    atrophy and fasiculations, hypotonia
  • Sensory normal
  • Deep Tendon Reflexes preserved until late in the
    disease
  • Pathologic Reflexes none

37
Scotts 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.

38
SpinoCerebellar Ataxia (SCA4)
  • Prior to Scotts 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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