Localization in the Neuraxis. The Approach to a Patient with Neurologic Disease zThe H&P accurately...
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Localization in the Neuraxis
The Approach to a Patient with Neurologic Disease
The H&P accurately localizes most lesionDivisions of the neuraxis have
specialized functionsDamage to various divisions produce
unique clinical deficitsLocalization is important
investigation modalities differ widely depending upon the level affected
Divisions of the Neuraxis
Cortical BrainSubcortical
BrainBrainstemCerebellumSpinal CordRootPeripheral
Nerve
Neuromuscular Junction
Muscle
Neurologic Examination
Higher Cortical FunctionCranial NervesCerebellar FunctionMotor SensoryDeep Tendon ReflexesPathologic Reflexes
Divisions of the Neuraxis
Cortical BrainSubcortical
BrainBrainstemCerebellumSpinal CordRootPeripheral
Nerve
Neuromuscular Junction
Muscle
Cortical Brain
Depends upon hemispheric dominanceNon-neurologists generalize:
right: visual/spatial, perception and memory
left: language and language dependent memory
Through detailed examination, neurologists should lateralize and localize within a lobe
Cortical Brain
Frontal Lobe: L:
Broca’s Aphasia
R: ? B:
precentral gyrus: motor homunculoussupplementary motor cortex: eye and head
turnprefrontal cortex: personality, initiativeparacentral lobule: cortical inhibition of
voiding B/B
Cortical Brain
Parietal Lobe: R:
anosognosia: left hemineglectdressing and constructional apraxiageographic agnosia
L:Gerstman’s Tetrad (not triad): L/R confusion,
finger agnosia, acalculia, agraphia without alexia
Werneke’s Aphasia
Cortical Brain
Parietal Lobe: B:
abnormal posture and passive movementlocalization of touch2-point discriminationastereognosisperceptual rivalry
Cortical Brain
Temporal: R:
hearing language
L:hearing sounds, rhythm, rhythm, music
B:learning and memory: mid/inferior gyriolfaction: limbicAuditory cortex: Heschel’s gyrus
Cortical Brain
Occipital Lobe: R:
micropsiamacropsia
B:visual hallucinations: elemental and unformedprosopagnosia: familiar facescortical blindness: striate cortices, normal pupil rxAnton’s: (para)striate, denial of obvious blindnessBalint’s: inability to direct voluntary gaze with
visual agnosia
Neurologic Examination when Cortical Brain is
Lesioned
Higher Cortical Function aphasia, apraxia, agnosia
Cranial Nerves: normal, unless forced eye deviation Cerebellar Function: normal Motor:
weakness of face/arm>leg (or vice versa) if motor homunculous is hit hypertonia if corticospinal tracts are hit
Sensory: sensory abn of face/arm>leg (or vice versa)
Deep Tendon Reflexes: hyper-reflexia
Pathologic Reflexes: Babinski’s reflex if corticospinal tracts are hit Frontal release signs (nonspecific), possibly Kernig and/or Brudzinski
Divisions of the Neuraxis
Cortical BrainSubcortical
BrainBrainstemCerebellumSpinal CordRootPeripheral
Nerve
Neuromuscular Junction
Muscle
Subcortical Brain
Deep white radiating fibers produce equal involvement of face/arm/leg weakness sensory abnormalities
Visual radiating fibers: (know how visual abnormalities morph with lesions from anterior to posterior brain)
deep parietal: bilateral homonomous quad on the floor
deep temporal (Meyer’s loop): bilateral homonomous quad in the sky
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 hypertonia
Sensory: sensory abnormalities in face=arm=leg
Deep Tendon Reflexes: hemi-hyper-reflexia
Pathologic Reflexes: Babinski’s reflex if corticospinal tracts are lesioned frontal release signs (nonspecific)
Divisions of the Neuraxis
Cortical BrainSubcortical
BrainBrainstemCerebellumSpinal CordRootPeripheral
Nerve
Neuromuscular Junction
Muscle
Brainstem
CN symptoms characterize BS diseaseThe Brainstem is basically spinal cord
with embedded cranial nerves cause symptoms of spinal cord disease, also Long Tract signs: (bilateral and crossed)
corticospinal (pyramidal): motorspinothalamic: pain/temp to the thalamusdorsal columns: prioprioception/vibration to thal.(due to decusation of long tracts, BS lesions do not produce
horizontal motor/sensory levels as in the cord, but rather vertical levels of hemiparesis/hemidysesthesias)
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: usually normal Motor: hemi-paresis (may be crossed), hemi-hypertonia,
spasticity Sensory: hemi-dysesthesias (may be crossed) Deep Tendon Reflexes: hemi-hyper-reflexia, brisk jaw jerk Pathologic Reflexes: Babinski’s reflex
Divisions of the Neuraxis
Cortical BrainSubcortical
BrainBrainstemCerebellumSpinal CordRootPeripheral
Nerve
Neuromuscular Junction
Muscle
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
Neurologic Examination when the Cerebellum is
Lesioned
Higher Cortical Function: normal Cranial Nerves: usually normal Cerebellar Function:
nystagmus flaccid dysarthria
Motor: normal bulk and strength with ipsilateral hemi-hypotonia intention worse than positional ipsilateral tremor axial instability with dysmetria
Sensory: normal Deep Tendon Reflexes: normal Pathologic Reflexes: normal
(plantar flexing to plantar stimulation)
Divisions of the Neuraxis
Cortical BrainSubcortical
BrainBrainstemCerebellumSpinal CordRootPeripheral
Nerve
Neuromuscular Junction
Muscle
Spinal Cord
Sensory levelSpasticity/hypertoniaWeakness:
extensors worse than flexors distal > proximal
Bowel and Bladder involvement: retention comes first, then detrusor
hyperactivity(both produce incontinence)
Neurologic Examination when the Spinal Cord is
Lesioned
Higher Cortical Function: normal Cranial Nerves: normal Cerebellar Function: normal Motor:
weakness (extensors worse than flexors) below the lesion para-hypertonia below the lesion with spasticity
Sensory: horizontal level usually lower than the lesion, poorly localizing may be somewhat assymetric
Deep Tendon Reflexes: para-hyper-reflexia below the level, possibly clonus
Pathologic Reflexes: loss of superficial reflexes (Beavor’s sign, cremasteric, anal
wink, etc) Babinski’s reflex
Divisions of the Neuraxis
Cortical BrainSubcortical
BrainBrainstemCerebellumSpinal CordRootPeripheral
Nerve
Neuromuscular Junction
Muscle
Root/Radiculopathy
Pain is the hallmark of a radiculopathy Sensory abnormalities in a dermatome provocative maneuvres exacerbate sharp, stabbing, hot, electric, radiating
Weakness in a myotome (assymetric) proximal (C5C6) distal (L5S1)
Neurologic Examination when a Root is Lesioned
Higher Cortical Function: normal Cranial Nerves: normal Cerebellar Function: normal Motor:
assymetric weakness, atrophy, and fasiculations in a myotome tone should be normal, unless multiple roots are severed
Sensory: assymetric dysesthesias confined to a dermatome anesthesia requires >1 root transection
Deep Tendon Reflexes: hypo- to a-reflexia if the root carries a reflex
Pathologic Reflexes: Spurling’s sign dural tension signs may be present (straight leg, crossed
straight leg, reverse straight leg, etc)
Divisions of the Neuraxis
Cortical BrainSubcortical
BrainBrainstemCerebellumSpinal CordRootPeripheral
Nerve
Neuromuscular Junction
Muscle
Peripheral Nerve(presuming nonfocality)
Weakness: distal predominant, (a)symetric
Sensory Dysesthesias: distal predominant
Autonomic involvement may occurTrophic changes: smooth shiny skin,
vasomotor abnormalities (edema, temperature dysregulation, vascular flushing), hair loss, nail changes
Neurologic Examination with Diffuse PN Lesioning
Higher Cortical Function: normal Cranial Nerves:
may be abnormal (know which peripheral CN’s associate with specific diseases)
Cerebellar Function: normal Motor: weakness is distal predominant if the PN is diffuse
atrophy, fasiculations, (hypotonia) Sensory:
dysesthesias, anesthesias, hyperpathia, allodynia, etc Deep Tendon Reflexes:
distal predominant hypo- to a-reflexia Pathologic Reflexes:
mute responses to plantar stimulation
Divisions of the Neuraxis
Cortical BrainSubcortical
BrainBrainstemCerebellumSpinal CordRootPeripheral
Nerve
Neuromuscular Junction
Muscle
Neuromuscular Junction
Fatiguability is the hallmarkWeakness: proximal and symmetric
exacerbated with use, recovers with rest
often affects facial muscles (ptosis, dysconjugate gaze, slack jaw)
muscles have normal bulk and toneSensation: preserved
Neurologic Examination in Disorders of the NMJ
Higher Cortical Function: normal Cranial Nerves:
fatiguability in ptosis, dysconjugate gaze, slack jaw Cerebellar Function: normal Motor:
fatiguable proximal weakness in both UE’s and LE’s no atrophy or fasiculations tone may be slightly decreased
Sensory: normal, though may complain of lowback pain
Deep Tendon Reflexes: may be hypo- to a-reflexic in LEMS may be normal in MG
Pathologic Reflexes: none
Divisions of the Neuraxis
Cortical BrainSubcortical
BrainBrainstemCerebellumSpinal CordRootPeripheral
Nerve
Neuromuscular Junction
Muscle
Muscle
Weakness of proximal arm and leg muscles symmetric
Sensation is normal though patients complain of cramping,
aching, and atrophy
Neurologic Examination in Disorders of Muscle
Higher Cortical Function: normal Cranial Nerves:
ptosis, dysconjugate gaze, slack jaw, bow-string lip, myopathic facies, dysphagia, dysphonia, (dysarthria)
Cerebellar Function: normal Motor:
usually proximal weakness in both UE’s and LE’s atrophy and fasiculations diffuse hypotonia accentuated primary and secondary curvature, scoliosis
Sensory: normal, though may complain of lowback pain
Deep Tendon Reflexes: preserved until late in the disease Pathologic Reflexes: ? Myotonia or cramping
Just a Few Things to Remember
Not all aphasias and apraxias are cortically based thalamus
The absence of Babinski’s reflex does not imply a lesion distal to the cord basal ganglia thalamus cerebellum
Compromised attention span results from lesioning: brain stem and RAS diencephalon: both sides bilateral cerebral hemispheres
Just a Few Things to Remember
Some neurologic diseases hit more than one level in the neuraxis
The tempo of progression allows one to narrow a differential diagnosis remarkably well . . . Always always always clarify this issue with the patient
Parsimony rulesNever fabricate part of the exam for
sake of being “thorough”
Just a Few Things to Remember
If you do not think of a complete differential diagnosis, you can not expect to catch the interesting diagnoses.
You must think of the possibile accademic diagnoses at this point in your career.
Patients pay you to rule out the worst firstWhen you are unsure of a diagnosis, it is
important to communicate this to patients and other physicians.