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Peripheral Neuropathy

guidelines

most of neuropathy is really CIDP.


 
Figure 5. Pes cavus
High-arched foot results from a chronic imbalance of intrinsic foot muscles in Charcot-Marie-Tooth disease.


  
Table 1.  Mono- and Generalized Neuropathies Commonly Encountered in Clinical Practice
Mononeuropathies Generalized neuropathies
Cranial neuropathies:
  • Trigeminal neuralgia
  • Bells palsy
  • Diabetic cranial neuropathies

Carpal tunnel syndrome (median nerve)
Cubital tunnel syndrome (ulnar nerve) Sciatic nerve damage following hip replacement
Diabetic mononeuropathy/mononeuropathy multiplex
Amyloidosis
Diabetic neuropathies:
  • Generalized sensorimotor polyneuropathy
  • Autonomic neuropathy
  • Polyradiculopathy

Hereditary neuropathies:
  • Charcot-Marie-Tooth disease
  • Amyloid polyneuropathy
  • Dejerine-Sottas disease
  • Refsums disease
Inflammatory/autoimmune neuropathies:
  • Guillain-Barré syndrome (GBS)
  • Chronic inflammatory demyelinating polyneuropathy (CIDP)
Toxic/metabolic/infectious diseases:
  • Various toxin-induced neuropathies
  • Alcoholic neuropathy
  • HIV neuropathy
  • Lyme neuropathy
  • Uremia
  • Porphyria
Systemic/malignancy-related neuropathies:
  • Paraneoplastic
  • Dysproteinemia
Table 7.  Key Findings from History and Physical Examination: Pattern Recognition for Generalized Neuropathies
Pattern Classification/possible diagnoses Comments
Symmetric proximal and distal weakness with sensory loss Inflammatory demyelinating polyneuropathy
Guillain-Barré syndrome (GBS)
Chronic inflammatory demyelinating polyneuropathy
Patient complaints include: difficulty raising arms to brush teeth and comb hair, problems climbing stairs, difficulty rising from a seated position, sensory loss, and sensory ataxia
Symmetric distal weakness with sensory loss Metabolic disorders (e.g., amyloidosis, diabetes, Sjögrens syndrome)
Drugs (amitriptyline, chloroquine, dapsone) and toxins
Hereditary neuropathies (Charcot-Marie-Tooth disease, amyloidosis)
Underlying conditions may be axonal or demyelinating;
therefore, laboratory investigations (e.g., fasting blood sugar, hemoglobin
A1C, vitamin B12 levels, molecular genetic blood tests) are recommended based on initial suspicions
Asymmetric distal weakness with sensory loss Vasculitis
Hereditary neuropathy with predisposition to pressure palsies
Infectious diseases (leprosy, Lyme, HIV)
Sarcoidosis
Compression and entrapment neuropathies
Vasculitis is the most common diagnosis when multiple nerves not normally subject to compression are involved. Compressive mononeuropathy, radiculopathy should be considered with single nerve/region involvement.
Asymmetric distal weakness without sensory loss Upper-motor-neuron disease
Generalized: multifocal motor neuropathy (MMP)
Bulbar findings (i.e., slurred or nasal speech, drooling, nasal regurgitation of liquids, difficulty whistling, swallowing), fasciculations,and hyperreflexia suggest upper-motor-neuron disease, and are rare with MMP
Asymmetric proximal and distal weakness with sensory loss Polyradiculopathy
Plexopathy (brachial or lumbar)
Meningeal carcinomatosis or lymphomatosis
In diabetic plexopathy, pain typically precedes weakness
Symmetric sensory loss without weakness Cryptogenic sensory polyneuropathy (CSPN)
Metabolic derangements (diabetes, alcohol), drugs, toxins
CSPN is primarily observed in older adults. Diabetic and alcoholic neuropathies may present with few or no motor signs.
Asymmetric proprioceptive sensory loss without weakness Sensory neuropathy (ganglionopathy)
Paraneoplastic syndrome
Sjögrens syndrome
Idiopathic sensory neuropathy
Drug (e.g., cisplatin and its analogues) or vitamin B6 toxicity
HIV sensory neuropathy
Primarily affects large fibers. May warrant a cancer workup.
Autonomic symptoms Generalized - diseases affecting small fibers: e.g., acute dysautonomia, familial/ primary amyloidosis, GBS, diabetes, Chagas disease, porphyria, HIV-related autonomic neuropathy, idiopathic pandysautonomia Typically associated with other types of neuropathy. Conduct autonomic testing (e.g., Valsalva heart rate response to pressure changes). May be an early or sole presentation of alcoholic or diabetic neuropathy.
Based on Barohn [6], Kowalske [13].

 
Table 8.  Assessment of Muscle-Group Weakness
Symptoms Likely source of weakness
Slurred/nasal speech
Drooling
Nasal regurgitation of liquids
Difficulty whistling, smiling
Difficulty swallowing, weight loss
Bulbar muscles
Diplopia
Ptosis
Extraocular muscles
Trouble reaching
Difficulty holding razor, comb, hair dryer
Difficulty placing things in high cabinets
Proximal upper-extremity muscles
Difficulty opening jars, doors, using keys, silverware
Trouble buttoning clothing
Distal upper-extremity muscles
Trouble rising from chairs, sofas
Trouble getting out of car, bath, or off toilet
Difficulty climbing stairs
Proximal lower-extremity muscles
Tripping
Sprained ankles
Distal lower-extremity muscles

 
Table 9. Principal Motor Innervation of Peripheral Nerves: Localizing Muscle Weakness
Nerves Muscles Action
Axillary Deltoid Shoulder abduction
Musculocutaneous Biceps, brachialis Flexion of elbow
Median Flexor carpi radialis Radial flexion of wrist
Flexor digitorum sublimis Flexion of middle phalanges (digiti II-V)
Flexor digitorum profundus (lateral half) Flexion of distal phalanges (digiti II, III)
Pronator teres, pronator quadratus Pronation of forearm
Abductor pollicis brevis Abduction of thumb
Opponens pollicis brevis Opposition of thumb
Flexor pollicis longus Flexion of distal phalanx of thumb
Flexor pollicis brevis Flexion of proximal phalanx of thumb
Ulnar Flexor carpi ulnaris Ulnar flexion of wrist
Flexor digitorum profundus (medial half) Flexion of distal phalanges (digiti IV, V)
Abductor digiti minimi Abduction of digiti V
All other intrinsics of hand Finger abduction/adduction
Radial Triceps Extension at elbow
Brachioradialis Flexion of forearm
Extensor carpi radialis/ulnaris Extension at wrist with radial/ulnar deviation
Supinator Supination of forearm
Extensor pollicis brevis Extension of thumb (proximal)
Extensor pollicis longus Extension of thumb (distal)
Extensor indicis proprius Extension of index (proximal)
Extensor digiti V proprius Extension of little finger (proximal)
Extensor digiti communis Extension of digits (II-V, proximal)
Femoral Iliopsoas Flexion of thigh at hip
Quadriceps Extension of leg at knee
Obturator Adductor longus, adductor brevis, adductor magnus Adduction of thigh at hip
Superior gluteal Gluteus medius, gluteus minimus, gluteus maximus Abduction of thigh at hip
Sciatic Biceps femoris, semitendinosus, semimembranosus Flexion of leg at knee
Sciatic branches: fibular (deep) Tibialis anterior Dorsiflexion of foot
Extensor digitorum longus Extension of toes
Extensor hallucis longus Extension of great toe
Sciatic branches: fibular (superficial) Peroneus Everts foot
Tibial Gastrocnemius, soleus Plantar flexion of foot
Flexor digitorum longus Flexion of distal phalanges (II-IV)
Flexor hallucis longus Flexion of distal phalanges (I)
Flexor digitorum brevis Flexion of middle phalanges (II-V)
Flexor hallucis brevis Flexion of middle phalanges (I)
Pudendal Perineal and sphincters Closure of sphincters, contraction of pelvic floor
From Ronthal [14].
 



 

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