Weakness

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Weakness

• Supischa Theerasasawat

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Content

• Hemiplegia
• Monoplegia
• Paraplegia
• Proximal weakness
• Distal weakness

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Hemiplegia

• Cortical lesion
• Subcortical lesion
• Brainstem lesion
• Spinal cord lesion

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Cerebral lesion
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Cortical lesion
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Cortical lesion

• more focal weakness than the weakness seen with
  subcortical lesions
• signs of cotical dysfunction

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Nature

• Vascular abrupt onset
• Epilepsy progression over seconds to minutes
• Migraine - progression over seconds to minutes
• Demyelinating disease - progression over days
• Mass (tumor or abscess) - progression over weeks

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Vascular causes

• MCA contralateral hemiparesis, predominate arm
  hand and face, with signs of cotical dysfunction
  (Rt. Neglect, Lt. aphasia)
• ACA - contralateral hemiparesis predominate leg,
  bowel bladder involvement
• PCA - contralateral hemianopia with memory deficit

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Subcortical lesion

• Equal weakness of the face, arm, and leg on the
  contralateral side

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Nature

• Vascular
• Lenticulostriate arteries - contralateral
  hemiparesis with little or no sensory involvement
• Thaiamoperforate arteries - contralateral sensory
  disturbance and movement disorders
  (chorcoathetosis or hemiballismus)

• Demyelinating disease
• Multiple Sclerosis - a history of episodes of
  relapsing and remitting neurological deficits
• Parainfectious Encephalomyelitis
• Progressive Multifocal Leukocnccphalopathy
  visual loss and weakness, immunocompromise hosts

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Brainstem lesion
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Brainstem lesion
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Spinal cord lesion

• hemiplegia sparing the face

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Spinal cord lesion

• Spinal Hemisection (Brown-Sequard's) Syndrome
  ipsilateral weakness, posterior column and
  contralateral pain and temperature
• Spinal Cord Compression - lower motor neuron
  (LMN) weakness at the level of the lesion and CST
  signs below the level of the lesion
• Spinal Cord Infarction spare posterior column

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Functional Hemiplegia

• Improvement in strength with coaching
• Inconsistencies in examination
• Hoover's sign
• Paralysis in the absence of other signs of
  motor system dysfunction, including tone and
  reflex changes

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Monoplegia

• Cortical lesion
• Spinal cord lesion
• Peripheral lesion
• Neuronopathies
• Radiculopathies
• Plexopathies
• Neuropathies

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Cortical lesion

• Vascular
• Epilepsy
• Migraine - the headache phase typically begins as
  the neurological deficit is resolving
• Demyelinating disease
• Mass

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Spinal cord lesion

• the weakness is associated with muscle wasting
  and lost reflexes in a radicular distribution

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Neuronopathies

• Weakness without sensory loss
• Monomelic amyotrophy progressive weakness over
  months to years
• Poliomyelitis acute asymetrical weakness

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Radiculopathies
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Plexopathies

• Pain
• Plexitis C5C6
• Neoplastic plexus infiltration C8T1
• Diabetic amyotrophy femoral nerve distribution

• No pain
• Radiation plexopathy upper brachail plexus
• Truama
• upper brachail plexus Erbs palsy
• lower brachail plexus Klumpkes palsy
• Thoracic outlet syndromes - lower brachail plexus
• Plexus hematoma lumbosacral plexus

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Peripheral nerves of the arms
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Peripheral nerves of the legs
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Paraplegia and spinal cord disease
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• Intramedullray lesion- suspended sensory loss,
  dissociative sensory loss, sacral sparing, early
  bowel bladder involvement
• Extramedullary lesion late bowel bladder
  involvement
• Intradural lesion
• Extradural lesion local pain

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Spinal Shock

• total loss of motor and sensory functions below
  the level of the lesion
• Temporary loss of spinal reflex below the level
  of lesion

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Unilateral Transverse Lesion

• Ipsilatcral weakness and loss of position and
  vibration below the level of the lesion
• Contralateral loss of pain and temperature caudal
  to the lesion

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Central Cord Syndrome

• Loss of sensation in the upper extremities of a
  dissociated type, with loss of pain and
  temperature sensation and preservation of
  position and vibration sensation

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Anterior Spinal Artery Syndrome

• Paraplegia, pain and temperature sensory loss
• Spared posterior column

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Foramen Magnum

• Occipital or neck pain
• Downbeat nystagmus, impaired sensation over the
  upper face, dysarthria, dysphonia, dysphagia
• Motor system involvement - spastic weakness
• Begins in the ipsilateral arm and is loll owed by
  weakness of the ipsilateral leg, spreading to the
  contralateral leg and then the arm

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Upper Cervical Spine

• Same as foramen magnum
• Radicular pain
• C2 - posterior of scalp
• C3 C4 - top of shoulder

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Lower Cervical and Upper Thoracic Spine

• Lesions at the C4-C6 level, pain and sensory
  disturbances - the radial aspect of the arm,
  forearm, and thumb
• Lesions at the C7-T1 level, pain and sensory
  disturbances - the ulnar aspect of the arm,
  forearm, and thumb
• Hoffmanns sign

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Thoracic Levels

• paraplegia
• the nipple (T4)
• the umbilicus (T10)
• the inguinal ligament (LI)

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Differentiation of conus lesions from cauda
equina lesions
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Differential diagnosis of diseases affecting the
spinal cord (compressive)

• Non-neoplastic
• Trauma
• Spondylosis
• Intervertebral disc herniation
• Spinal stenosis
• Infectious disorders (e.g., abscess,
  tuberculosis)
• Inflammatory (e.g., rheumatoid arthritis,
  ankylosing spondylitis, sarcoid)
• Spinal hemorrhage
• Syringomyelia
• Congenital disorders
• Arachnoid cysts
• Paget's disease
• Osteoporosis

• Neoplastic
• Epidural
• Intradural extramedullar (e.g., meningioma,
  neurofibroma, and leptomeningeal metastasis)
• Intramedullary

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Noncompressive myelopathy

• Demyelinating (e.g., multiple sclerosis, acute
  disseminated encephalomyelitis)
• Viral myelitis (e.g., roster, acquired
  immunodeficiency syndrome-related myelopathy,
  human T-lymphotropic virus type 1)
• Vitamin B12 deficiency and other nutritional
  deficiencies
• Infarction
• Ischemia and hemorrhage resulting from vascular
  malformations
• Spirochetal diseases (syphilis and Lyme disease)
• Toxic myelopathies (e.g., radiation induced)
• Autoimmune diseases (e.g., lupus, Sjogren's
  syndrome)
• Paraneoplastic
• Neuronal degenerations
• Acute and subacute transverse myelitis of unknown
  cause

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Proximal and distal weakness
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Proximal weakness

• NMJ lesion - fluctuation
• Myopthy no fluctuation except metabolic myopathy

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Gowers sign
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Distal weakness

• Neuropathy
• Distal myopathy

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Fluctuating weakness

• NMJ lesion
• Myasthenia gravis
• Lambert-Eaton myasthenic syndrome
• Periodic paralysis
• Metabolic abnormalities
• Defect of glycogen metabolism
• Defect of lipid metabolism
• Defect of mitochondria metabolism

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Lambert-Eaton myasthenic syndrome

• anti-voltage-gated calcium channel antibodies
• Weakness of the shoulder and especially the hip
  girdle predominates
• Sparing the bulbar, ocular, and respiratory
  muscles
• Reflexes are typically reduced or absent at test.
  After a brief exercise, weakness and reflexes are
  often improved

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Periodic paralysis

• provoked by rest after exercise
• totally paralyzed
• sparing bulbofacial muscles and rarely
  respiratory muscles involvement

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Defect of glycogen metabolism

• Fatigue and muscle pain in the first few minute

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Defect of lipid metabolism

• Weakness after exercise 30-60 min

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Defect of mitochondria metabolism

• particular weakness of the extraocular
• resting tachycardia
• high lactic acid levels in the blood
• excessive sweating

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Acquired Disorders Causing Weakness

• Motor neuron diseases
• Inflammatory myopathy
• Toxic myopathy
• Endocrine disorders of muscle
• Neuromuscular transmission disorders
• Peripheral motor neuropathies

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Motor neuron diseases

• ALS
• Kennedys disease

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Inflammatory myopathy

• Dermatomyositis
• Polymyositis
• Inclusion body myositis

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Toxic myopathy

• Alcohol
• Other drugs steriods, statin, colchicine

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Endocrine disorders of muscle

• Thyriod myopathy

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Neuromuscular transmission disorders

• Myasthenia gravis
• Lambert-Eaton myasthenic syndrome

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Peripheral motor neuropathies

• Drug-induce dapsone, vincristine
• AIDP
• Multifocal motor neuropathy with conduction block

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Lifelong nonprogressive disorders

• Early childhood
• Slender dysmorphic with diffuse weakness

• Congenital muscular dystrophy
• central core disease
• nemaline myopathy
• congenital fiber-type disproportion

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Lifelong progressive disorders

• Anterior horn cells - the spinal muscular
  atrophies
• Peripheral motor nerve - Charcot-Marie-Tooth
  polyneuropathies
• Muscle - muscular dystrophies

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Disorders with Prominent Ocular Weakness

• Oculopharyngeal muscular dystrophy
• slowly progressive weakness of the eye muscles -
  ptosis and external ophthalmoplegia
• difficulty in swallowing.

• The Kearns-Sayre syndrome
• Ptosis
• extraocular muscle palsies
• pigmentary degeneration of the retina
• cerebellar ataxia
• pyramidal tract signs
• short stature
• mental retardation
• cardiac conduction defects

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Disorders with Distinctive Facial or Bulbar
Weakness

• FSH muscular dystrphy
• Myotonic dystrophy type I

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FSH muscular dystrphy

• Early adult
• Asymmetrical weakness
• Weakness of the face ? difficulty with whistling
  or blowing up balloons
• Weakness of the muscles around the shoulders is
  always seen, deltoid muscle spared

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Myotonic dystrophy type I

• Predominantly distal weakness
• The neck flexors and temporal and masseter
  muscles are often wasted
• The long, thin face with hollowed temples,
  ptosis, and frontal balding
• Percussion myotonia and grip myotonia

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Disorders with Distinctive Shoulder-Girdle or Arm
Weakness

• Emery-Dreifuss muscular dystrophy
• Distal muscular dystrophies
• myotonic dystrophy
• Welander's myopathy

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Disorders with Prominent Hip-Girdle or Leg
Weakness

• SMA
• Inherited muscular dystrophies
• Duchenne's muscular dystrophy
• Becker's muscular dystrophy
• LGMD

• Inflammatory myopathy
• Distal myopathy
• Markesbcry-Griggs Udd myopathy
• Nonaka myopathy
• Laing myopathy
• Miyoshi myopathy