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Goals:
1. Learn how basic science anatomy and physiology applies to understanding neuropathy.
2. Learn the patterns of disease by which peripheral neuropathy presents clinically.
3. Learn a simple approach to diagnose peripheral neuropathy at the bedside.
Reading:
Cecil Essentials of Medicine, 7th Ed. 2007 p1146-51
Further reading for reference:
Hughes, RAC 2008 Prac Neurol 8: 396-405
Hughes, RAC 2002 BMJ 324:466-9
Poncelet, AN 1998 Am Fam Phys 57:755-764
Donofrio, PD & Albers, JW.1990 Muscle & Nerve 13:889-903
Lecture Notes: The Key Points.
A useful approach to neurology clinical problem solving is to ask and answer the
following questions in sequence:
1) Where is the lesion?
2) What is the cause of the lesion?
3) What is the treatment of the lesion?
The approach is used in the seminar. Peripheral neuropathy is a common condition and
encountered in a whole host of medical subspecialities, not just the neurology service,
and the reason is because it has so many causes.
Bedside Question 1. Where is the lesion?
The answer hinges on knowing the anatomy of peripheral nervous system and how injury
causes symptoms and signs. The peripheral nervous system consists of root, plexus and
peripheral nerve. Each of these potential localizations causes a signature clinical pattern
which can be deduced at the bedside. Root injury (radiculopathy) produces motor and
sensory deficits in a segmental distribution, plexus injury (plexopathy) in the distribution
of its organizational anatomy and peripheral nerve injury in a particular nerve
distribution. Setting radiculopathy and plexopathy aside, disease can either affect the
nerve cell body, also called neuronopathy (which in the case of motor neurons is called
motor neuron disease and for sensory neurons is called sensory ganglionopathy), or the
peripheral process (which is by convention referred to as peripheral neuropathy).
Peripheral nerve injury (peripheral neuropathy) results when nerves are diseased either:
1) focally (called mononeuropathy) like carpal tunnel syndrome, as may be seen in
pregnancy with fluid retention, or
2) focally but multiply (called mononeuritis multiplex) for example in vasculitis,
Sensory
Large Fiber
Sensory
Small Fiber
Paresthesias
Dysesthesias
Allodynia
Autonomic
nerves
Sweating
Hypotension
Urinary retension
Impotence
Vascular color changes
Sweating
Hypertension
1. DURATION OF NEUROPATHY?
Most polyneuropathies are chronic i.e. months and years
For acute polyneuropathies think
Guillain Barre syndrome
Vasculitis
Toxins and Drugs
Tick paralysis
Diphtheria
Porphyria
If relapses and remissions think
Intermittent toxin exposure
Chronic Inflammatory Demyelinating Polyneuropathy
2. DISTRIBUTION OF NEUROPATHY DEFICITS?
If predominant motor fibers think:
Motor neuron disease (motor neuronopathy) exclusively motor
Multifocal motor neuropathy exclusively motor
Acute Inflammatory Demyelinating Polyneuropathy (Guillain-Barre)
Chronic Inflammatory Demyelinating Polyneuropathy
Lead intoxication
Acute porphyria
Charcot-Marie-Tooth disease (HMSN)
If pure sensory/ severe proprioceptive deficit, think sensory neuronopathy:
Carcinoma (paraneoplastic)
Sjogrens syndrome
Drugs like Cisplatinum, styrene, B6 toxicity
HIV
Friedrichs ataxia
If autonomic nerves involved (small fiber) think:
Diabetes
Amyloid
Drugs like Vincristine, ddI, ddC
HIV
Hereditary Sensory Autonomic Neuropathy (HSAN)
Guillain-Barre
Acute porphyria
4. DISEASE PATHOLOGY?
If demyelination and uniform, cause is hereditary. Think of:
Charcot-Marie-Tooth disease (HMSN)
Hereditary Neuropathy with Liability to Pressure Palsy
Rarer myelin disorders like adreno- and metachromatic leukodystrophy
Friedrichs ataxia
If demyelination is non uniform the cause is acquired. Think of:
Acute Inflammatory Demyelinating Polyneuropathy
Chronic Inflammatory Demyelinating Polyneuropathy
Monoclonal gammopathy
Acute arsenic poisoning
Friedrichs ataxia
Axonal polyneuropathies are by far more common than demyelinating forms. If otherwise
unremarkable however length dependent, chronic, sensory more than motor axonal
polyneuropathy without associated toxin, drug or disease consider cryptogenic sensory axonal
polyneuropathy. Exclude treatable diseases with the following five laboratory tests (no need for
antibody panels!): TSH (for hypothyroidism), HBA1c (for diabetes), B12 and methylmalonic acid
(for B12 deficiency), serum protein electrophoresis with immunofixation (for monoclonal
gammopathy) and creatinine (for uremia).