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Proximal and Distal Muscle Weakness

The document outlines the assessment and examination process for muscle weakness, detailing the history to gather, physical examination techniques, and potential causes for proximal and distal muscle weakness. It highlights various conditions associated with muscle weakness, including myopathies, motor neuron diseases, and neuropathies, along with suggested investigations like manual muscle testing and imaging. Additionally, it mentions overlap syndromes that can present with both types of weakness.

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ahmed naeem
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3 views

Proximal and Distal Muscle Weakness

The document outlines the assessment and examination process for muscle weakness, detailing the history to gather, physical examination techniques, and potential causes for proximal and distal muscle weakness. It highlights various conditions associated with muscle weakness, including myopathies, motor neuron diseases, and neuropathies, along with suggested investigations like manual muscle testing and imaging. Additionally, it mentions overlap syndromes that can present with both types of weakness.

Uploaded by

ahmed naeem
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PDF, TXT or read online on Scribd
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1.

History:
* Gather a detailed history, including:
* Onset and duration of weakness
* Symmetry and distribution of weakness
* Associated symptoms (e.g., pain, fatigue, difficulty breathing, swallowing, or
speaking)
* Previous surgeries or injuries
* Medications and supplements
* Family history of neuromuscular disorders

Additional Considerations:
* Age: Proximal weakness is more common in adulthood, while distal weakness
may occur at any age.
* Distribution: Symmetric (both sides) or asymmetric (one side) distribution can
provide clues to the underlying cause.
* Progression: Rapid progression suggests acute conditions (e.g., Guillain-Barré
syndrome), while slow progression is seen in chronic disorders (e.g., muscular
dystrophies).

2. Physical Examination:
* General Assessment: Observe overall muscle bulk and symmetry.
* Neurological Examination:
* Test muscle strength (manual muscle testing)
* Assess reflexes (deep tendon reflexes, plantar reflexes)
* Evaluate sensory function
* Perform cranial nerve examination
* Specific Muscle Group Examination: Assess strength and range of motion in
proximal (shoulders, hips, thighs) and distal (wrists, hands, feet) muscle groups.
Proximal Muscle Weakness
Typically indicates involvement of large muscle groups and may point towards
myopathies, motor neuron diseases, or neuromuscular junction disorders.
* Myopathies:
* Muscular dystrophy (e.g., Duchenne muscular dystrophy, Becker muscular
dystrophy)
* Polymyositis
* Dermatomyositis
* Inclusion body myositis
* Mitochondrial myopathies
* Neuromuscular Junction Disorders:
* Myasthenia gravis
* Lambert-Eaton syndrome
* Spinal Muscular Atrophy:
* Spinal muscular atrophy type 1 (Werdnig-Hoffmann disease)
* Spinal muscular atrophy type 2
* Spinal muscular atrophy type 3 (Kugelberg-Welander disease)
* Motor Neuron Diseases:
* Amyotrophic lateral sclerosis
* Primary lateral sclerosis
* Progressive bulbar palsy
* Spinal and bulbar muscular atrophy
* Other:
* Critical illness myopathy
* Steroid-induced myopathy
* Endocrine disorders (e.g., hypothyroidism, Cushing's syndrome)
* Metabolic disorders (e.g., Pompe disease, acid maltase deficiency)

Assessment:(investigations)
* Manual muscle testing (e.g., Gower's sign for proximal lower extremity
weakness)
* Gait analysis
* Functional tests (e.g., chair stand, stair climbing)
* Imaging: MRI, EMG/NCS

Distal Muscle Weakness


Usually suggests involvement of peripheral nerves and may be caused by
neuropathies or muscular dystrophies.
* Peripheral Neuropathies:
* Guillain-Barré syndrome
* Chronic inflammatory demyelinating polyneuropathy (CIDP)
* Diabetic neuropathy
* Alcoholic neuropathy
* Vitamin B12 deficiency neuropathy
* Charcot-Marie-Tooth disease
* Hereditary sensory and autonomic neuropathy
* Motor Neuron Diseases:
* Amyotrophic lateral sclerosis (can also cause distal weakness)
* Other:
* Hypothyroidism
* Vitamin E deficiency
* Lead poisoning
* Arsenic poisoning
* Mercury poisoning

Assessment:
* Manual muscle testing (e.g., finger abduction, toe flexion)
* Neurological examination (e.g., reflexes, sensation)
* Imaging: EMG/NCS
* Electrophysiological studies (e.g., nerve conduction velocity, repetitive nerve
stimulation)

Additional Tests:
* Blood tests: To rule out metabolic or immunologic disorders
* Genetic testing: To identify specific genetic mutations
* Biopsy: Muscle or nerve biopsy to confirm the diagnosis

Overlap Syndromes:
* Some neuromuscular disorders can cause both proximal and distal muscle
weakness, such as:
* Myotonic dystrophy
* Facioscapulohumeral muscular dystrophy
* Scapuloperoneal muscular dystrophy

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