1 APRIL 2022 ausdoc . com . au encountered metabolic / toxic neuropathies .
COMMON ENTRAPMENT NEUROPATHIES
THE MEDIAN , ulnar , radial and fibular nerves are most commonly affected by entrapment neuropathies . Early diagnosis and effective management are important for improving quality of life and preserving limb function . Neurophysiological studies form the cornerstone of diagnosis and enable stratification of nerve dysfunction with treatment directed to the individual level of impairment . Ancillary tests , including ultrasound of the nerve , aid confirmation and localisation . 50
Figure 3 . Left foot of a patient with autonomic neuropathy and a plantar diabetic foot ulcer . The dry skin is prone to cracking , fissuring , and ulceration as illustrated beneath the first metatarsal head .
Del Core MA et al . Foot & Ankle Orthopaedics . July 2018 ; Reprinted by Permission of SAGE Publication
Carpal tunnel syndrome
Median neuropathy ( or carpal tunnel syndrome ) resulting from median nerve compression at the wrist is the most common mononeuropathy in adults . 50
The prevalence of carpal tunnel syndrome may be significantly higher in certain occupations and among women . 51
Paraesthesia and pain affecting the thumb , index and middle fingers and the lateral aspect of the fourth finger is the classical presentation ( see figure 6 ). Patients may report sensory symptoms involving the entire hand and proximal radiation to the upper limb . Sensory symptoms are worse at night , often waking the patient , and are exacerbated by repetitive activities requiring wrist flexion .
Examination reveals sensory loss on the palmar aspect of the thumb , index and middle fingers and lateral fourth digit . 50
Motor symptoms indicate an advanced stage and include weak grip and difficulty performing fine motor tasks . The specific finding of selective weakness of abductor pollicis brevis and opponens pollicis with preserved strength in the flexor pollicis longus helps localise the pathology to the carpal tunnel . Tinel sign ( pain / paraesthesia on nerve percussion ) has a low sensitivity ( 30-43 %) and a specificity up to 65 %. 52 The Phalen test , performed by asking the patient to flex the wrists and press the dorsum of both hands together for 30-60 seconds , has a sensitivity of 50-67 %, and lower specificity ( 15-17 %) because of false positives from musculoskeletal pain hindering the interpretation . 53 , 54 A negative Phalen test ( no reproduction of symptoms ) is highly predictive of normal nerve conduction studies . 55
An increased median nerve cross-sectional area of more than 10mm 2 on ultrasound has a high sensitivity and specificity for carpal tunnel syndrome . 56
Treatment is stratified based on severity ( see table 5 ).
ACQUIRED IMMUNE DEMYELINATING NEUROPATHIES
IT IS important to recognise demyelinating polyneuropathies because , if detected in time , their immune-mediated aetiopathogenesis is often amenable to treatment . There is a specific set of clinical features that enables rapid clinical diagnosis ; further confirmation is with neurophysiological assessment and ancillary testing , including CSF examination . These conditions include subacute inflammatory demyelinating polyradiculoneuropathy ( SIDP , which progresses over 4-8 weeks ), chronic
Figure 2 . Distal symmetric polyneuropathy .
Table 4 . Metabolic and toxic axonal neuropathies
Aetiology Predominant pattern Affected fibres Evolution Comment
Alcoholic neuropathy |
Sensory |
Small fibres |
Chronic |
Small fibres affected in toxic alcoholic neuropathy |
|
|
|
|
and large fibres affected in metabolic alcoholic |
|
|
|
|
neuropathy from secondary vitamin deficiencies |
Uraemic neuropathy |
Sensory |
Large fibres |
Chronic |
Progression can be potentially reversed with |
|
|
|
|
dialysis |
|
|
|
|
Carpal tunnel syndrome is common |
Amyloid neuropathy Sensory , autonomic Small fibres Carpal tunnel syndrome is common
Porphyric neuropathy |
Motor predominant |
Large fibres |
Proximal weakness |
|
|
|
more common |
inflammatory demyelinating polyradiculoneuropathy ( CIDP , which progresses beyond eight weeks and has several clinical types ) and Guillain-Barré syndrome where progression occurs over up to four weeks . The distinction is important as the type of immunomodulation and response to immunomodulation varies . 57
Guillain-Barré syndrome
Guillain-Barré syndrome ( GBS ) is an acute polyneuropathy . It can occur at any age , from neonates to the elderly , with mean age of onset around 40 and a slight male preponderance . 57
Common presentations include initial sensory symptoms ( acral paraesthesia ) followed shortly by ascending motor symptoms . The latter are often symmetrical and progressive , peaking by 3-4 weeks after onset . 58 They may range from mild weakness to flaccid quadriplegia , and , in about a third of patients , respiratory failure . 58 Bifacial lower motor neuron weakness , present in almost half of patients , often helps clinch the diagnosis . A significant
GBS mimic
Thiamine deficiency |
Motor |
Large fibres |
Acute-subacute |
Systemic manifestations including cardiac |
|
|
|
|
symptoms and oculomotor palsy are common |
Vitamin B12 deficiency ( see figure 5 )
Sensory |
Large fibres |
Subacute-chronic |
Consider iatrogenic causes such as metformin and PPIs Symptoms may start simultaneously in hands and feet , especially in cases with myeloneuropathy |
Folate deficiency Sensory Large fibres Subacute-chronic Rarely occurs in isolation
Copper deficiency |
Sensory |
Large fibres |
Subacute-chronic |
Commonly presents as myeloneuropathy and |
|
|
|
|
closely mimics B12 deficiency-related syndrome |
Vinca alkaloids ( vinblastine , vincristine )
Figure 4 . Diabetic amyotrophy .
Sensorimotor |
Large fibres |
Acute-subacute |
Presence of diabetes further predisposes to |
|
|
|
development of neuropathy |
Isoniazid Sensorimotor Large fibres Acute-subacute Pyridoxine administration can be preventive
Heavy metals ( lead ) |
Motor predominant |
Large fibres |
Acute-subacute |
Upper limb involvement more common with wrist |
|
|
|
|
drop frequent |
Heavy metals ( arsenic , thallium ) Sensorimotor Large fibre Acute-subacute GBS / porphyria mimic
number of patients have radicular pain , although the sensory loss is usually mild . 58 There is a wide range of GBS variants with differing presentations .
Nerve conduction studies have varying sensitivity depending on their timing , ranging from 20 % at 1-2 weeks to 87 % at 4-5 weeks . 59
The neurophysiological features evolve from early prolongation of F-wave latencies , followed by prolongation of distal motor latencies and subsequently temporal dispersion with or without conduction blocks , and
Reproduced from BMJ Case Reports , Khan ZU , et al ; 14 : e239869 ,
2021 with permission from BMJ Publishing Group Ltd .
then a drop in velocities of nerve conduction . 59 It is important to be aware of both the sensitivity and evolution of neurophysiological findings to enable diagnosis based on clinical suspicion ; this enables early treatment despite neurophysiological findings not being supportive in the early stages of disease . Note that neurophysiological abnormalities can persist well beyond clinical recovery and do not indicate a diagnosis of CIDP .
Plasma exchange and IV immunoglobulin ( IVIg ) are the PAGE 48