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weakness_umn

upper motor neuron weakness

Introduction

  • muscle weakness due to pathology affecting the upper motor neurons which characteristically results in hyperreflexia, spasticity, clonus and muscle weakness
  • in a patient with decreased conscious state, roving eye movements suggest either:
    • bilateral lesions above the brainstem, or,
    • other causes of coma
  • the presence of horizontal myoclonus of the eyes (“eyes shaking”) with clonus of legs > arms suggests serotonin syndrome

Anatomy

  • UMN “pyramidal” fibres
    • NB. UMNs do not technically have “nuclei” in the classical sense of clustered lower motor neuron cell bodies, instead, their cell bodies form layers (like layer V pyramidal cells in the cortex) or reside within specific brainstem motor centers. They project their axons downward to synapse on lower motor neuron nuclei in the brainstem or spinal cord
    • cerebral cortex / pyramidal tracts:
      • these control voluntary, fine and skilled movements and pass through the medullary pyramids where the lateral corticospinal tract fibres decussate/cross over and consist of:
        • corticospinal tracts - synapse with spinal nerves
          • within the spinal cord they form:
            • the lateral corticospinal tract
              • 89-90% of all corticospinal fibres
              • decussate in the medullary pyramids
              • controls fine, precise, and skilled voluntary movements of the distal limbs (like fingers and hands)
            • the anterior corticospinal tract
              • do not cross in the brainstem, they descend ipsilaterally and cross over later at the specific spinal cord level where they synapse
              • controls voluntary movements of the axial and proximal muscles (the neck, trunk, and shoulders) to maintain posture
        • corticobulbar tract - synapse with cranial nerves
    • UMN “nuclei” locations
      • primary motor cortex:
        • located in the precentral gyrus (Brodmann area 4) of the frontal lobe
      • premotor cortex and supplementary motor area:
        • located just anterior to the primary motor cortex in the frontal lobe
      • other cortical areas:
        • portions also arise from the parietal lobe and postcentral gyrus
  • UMN brainstem / extrapyramidal tracts:
    • these are for involuntary, postural and autonomic controls and completely bypass the medullary pyramids
    • red nucleus:
      • located in the midbrain (giving rise to the rubrospinal tract)
    • vestibular nuclei:
      • located in the pons and medulla (giving rise to vestibulospinal tracts)
    • reticular formation nuclei:
      • located throughout the brainstem (giving rise to reticulospinal tracts)
    • superior colliculus:
      • located in the midbrain (giving rise to the tectospinal tract)
  • the activity of these circuits is strongly modulated particularly by the basal ganglia and substantia nigra
    • striatum
      • the striatum receives diverse inputs from the cortex, midbrain, hippocampus, and other regions, which it uses to coordinate planning, movement, and decision-making, as well as processing reward
      • the medium spiny neurons in the striatum are inhibitory neurons and respond to dopamine
        • most belong to either:
          • the direct pathway, which helps to promote movement and express dopamine receptor 1 (D1)
          • the indirect pathway, which suppresses unwanted movements and express dopamine receptor 2 (D2).
        • there appear to be 9 types of medium spiny neurons in the striatum including:1)
          • D1 outliers: show high expression of genes involved in addiction and substance use disorder, especially genes related to opioid response (the mu opioid receptor (OPRM1) )
          • D2 outliers: show high expression of genes that respond to antidepressants
        • role in Huntington's disease:
          • dorsal populations of medium spiny neurons express higher levels of the genes MSH2 and MSH3, which play a role in increasing the number of CAG repeats found in the huntington gene and a rare population of medium spiny neurons that forms island-like structures in the ventral striatum were more resistant to the accumulation of CAG repeats
  • the term supranuclear refers to parts of the CNS above the cranial nerve motor nuclei in the brainstem
    • if the actual cranial nerve motor nuclei are intact, reflex movements often still work
      • for example, in eye disorders, reflex movements like the vestibular-ocular reflex (doll's head maneuver) can bypass a damaged supranuclear area
    • bilateral supranuclear lesions affecting eye movements:
      • often impair voluntary control, such as the ability to intentionally look up, down, or side-to-side (saccades), even though involuntary reflex tracking might remain partially preserved
    • Progressive Supranuclear Palsy (PSP) is a neurodegenerative disorder causing balance issues, rigidity, and vertical gaze palsy

Aetiology

bilateral

  • cerebral palsy
  • traumatic brain injury
  • spinal cord injury
  • spinal cord ischaemia
  • transverse myelitis
  • auto-immune post-viral leukoencephalopathy (ADEM) - acute, rapidly progressive, mainly in children
  • pseudobulbar palsy - involves muscles innervated by the cranial nerves IX, X and XII, may have labile emotions and bilateral upper motor limb signs
  • decorticate posturing
    • occurs due to severe cerebral cortical dysfunction
    • results in arms are bent inward at the elbows and held tightly against the chest with wrists and fists clenched, while the legs are stiffly extended and rotated inward
  • decerebrate posturing
    • occurs due to severe brainstem dysfunction below the red nucleus
    • results in all four limbs are rigidly straightened and extended out, with the arms pronated (palms facing away), toes pointed downward, and the head and neck arched backward

unilateral

weakness_umn.txt · Last modified: 2026/09/04 03:06 by gary1

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