Peripheral Neuropathies Flashcards
Acquired peripheral neuropathies may arise from a variety of causes including metabolic, endocrine, infectious, immune-mediated, deficiency states, toxins, drugs, vascular, paraneoplastic manifestations, or idiopathic causes.
What are some common metabolic/endocrine causes of peripheral/polyneuropathies?
Diabetes mellitus
Thyroid disease
Uremia
Porphyria
Acquired peripheral neuropathies may arise from a variety of causes including metabolic, endocrine, infectious, immune-mediated, deficiency states, toxins, drugs, vascular, paraneoplastic manifestations, or idiopathic causes.
What are some infectious causes of polyneuropathies?
Mononucleosis Hepatitis Lyme disease HIV Leprosy Syphilis Herpes zoster
Acquired peripheral neuropathies may arise from a variety of causes including metabolic, endocrine, infectious, immune-mediated, deficiency states, toxins, drugs, vascular, paraneoplastic manifestations, or idiopathic causes.
What are some immune mediated causes?
GBS, CIDP, Miller Fisher
Multifocal motor neuropathy
Paraproteinemic (monoclonal gammopathy)
Acquired peripheral neuropathies may arise from a variety of causes including metabolic, endocrine, infectious, immune-mediated, deficiency states, toxins, drugs, vascular, paraneoplastic manifestations, or idiopathic causes.
What are some deficiency states that may play a role in developing a polyneuropathy?
B1, B6, B12, E, Copper
Acquired peripheral neuropathies may arise from a variety of causes including metabolic, endocrine, infectious, immune-mediated, deficiency states, toxins, drugs, vascular, paraneoplastic manifestations, or idiopathic causes.
What are some toxins that may cause peripheral neuropathy?
Alcohol
Metals: Pb, As, Hg, Th
Organic compounds: n-hexane, organophosphates
Acquired peripheral neuropathies may arise from a variety of causes including metabolic, endocrine, infectious, immune-mediated, deficiency states, toxins, drugs, vascular, paraneoplastic manifestations, or idiopathic causes.
What are some drugs that may cause polyneuropathy?
Vinca alkaloids (vincristine —> polyneuropathy in 100% of pts)
Phenytoin Isoniazid Amiodarone Cis-platinum Nitrofurantoin
_____ deficiency causes a peripheral neuropathy that affects both the corticospinal tracts (+babinski) and dorsal columns (lose DTRs, neuropathy) of the spinal cord
B12
Acquired peripheral neuropathies may arise from a variety of causes including metabolic, endocrine, infectious, immune-mediated, deficiency states, toxins, drugs, vascular, paraneoplastic manifestations, or idiopathic causes.
What are some conditions associated with vascular causes of peripheral neuropathy?
RA
SLE
Polyarteritis nodosa
Acquired peripheral neuropathies may arise from a variety of causes including metabolic, endocrine, infectious, immune-mediated, deficiency states, toxins, drugs, vascular, paraneoplastic manifestations, or idiopathic causes.
Of the above, ________ causes are associated with pure sensory neuropathy (dorsal ganglionopathy)
Paraneoplastic
Pure sensory polyneuropathies are rare. In these cases there are 2 classifications to be considered: Sensory ganglionopathy and small-fiber neuropathy
What are 2 underlying etiologies of a sensory ganglionopathy?
Paraneoplastic (search for occult malignancy)
Toxins (platinum based chemotherapeutics)
Pure sensory polyneuropathies are rare. In these cases there are 2 classifications to be considered: Sensory ganglionopathy and small-fiber neuropathy.
What are the clinical features of a small-fiber neuropathy in terms of pain, temperature, light touch, reflexes, and strength?
Pain and temperature affected
Light touch, proprioception, reflexes, and strength are preserved!
Signs and symptoms of small fiber polyneuropathy
Signs — decreased pin-prick and temp sensation, dysesthesias to light touch. Normal strength, reflexes, proprioception, vibratory sensation
Symptoms — pain, “burning” dysesthesias, paresthesias, temperature sensation abnormalities
What are EMG findings with small fiber polyneuropathy? How is the diagnosis confirmed?
EMG/NCV is normal!
Dx based on skin biopsy — shows decreased epidermal nerve fiber density
[EMG is normal because small fibers are unmyelinated, and EMG only detects abnormalities of myelinated fibers]
Most common identifiable cause of neuropathy in the US
Diabetes mellitus
What are the different forms of polyneuropathy seen in DM?
Distal symmetric sensorimotor neuropathy (“stocking and glove”) — most common
Cranial neuropathy (CN III, VI, VII)
Mononeuropathy (carpal tunnel, etc)
Mononeuropathy multiplex
Autonomic neuropathy
Lumbosacral plexopathy (diabetic amyotrophy)
Radiculopathy
Charcot Marie Tooth is a hereditary motor sensory neuropathy; what is the inheritance?
Autosomal dominant
Differentiate Hereditary Motor Sensory Neuropathy type I vs. type II in terms of major pathologic feature and clinical presentation
[HMSN = Charcot-Marie-Tooth]
Type I = Most common; Demyelinating is major feature. Onset 1st or 2nd decade; often see difficulty walking or running, distal symmetric atrophy, arreflexia, mild sensory loss, skeletal deformities (pes cavus, hammer toes, scoliosis)
Type II = Axonal loss is major feature. Onset in adulthood; distal symmetric atrophy, arreflexia, mild sensory loss
EMG findings with HMSN type I vs type II
Type I EMG — slowling of motor nerve conduction velocities (i.e., demyelination)
Type II EMG — normal or nearly normal motor nerve conduction velocities (i.e., axonal loss
Hereditary polyneuropathy caused by alpha galactosidase deficiency
Fabry’s disease
Hereditary polyneuropathy due to alrylsulfatase A deficiency
Metachromatic leukodystrophy
Hereditary polyneuropathy due to deficiency in HDL
Tangier disease
Refsum’s disease is a hereditary polyneuropathy also known as _______ _____storage disease
Phytanic acid
Hereditary polyneuropathy due to defect in heme biosynthesis
Porphyria
Globoid cell leukodystrophy, abetalipoproteinemia (bassen-kornzweig syndrome), and familial amyloid neuropathies are __________
A. Acquired polyneuropathies B. Acquired mononeuropathies C. Hereditary mononeuropathies D. Hereditary polyneuropathies E. None of the above
D. Hereditary polyneuropathies
Acute/subacute ascending motor paralysis often following a viral syndrome (EBV, mycoplasma pneumoniae, C.jejuni enteritis), surgery, or immmunization
Guillain-Barre syndrome
Clinical features of GBS
Low back/leg pain possible at onset
Ascending usually symmetric weakness
Hypo or absent DTRs
No/minimal sensory symptoms or signs
Possible respiratory failure; autonomic involvement
Key lab findings in GBS involving CSF and NCVs
CSF: albumino-cytologic dissociation (increased protein, normal cell count, normal glucose)
NCVs: slow conduction velocity, focal conduction block, prolonged F-waves
Treatment for GBS
General supportive care with attention to: swallowing, respiration, cardiovascular support, infection, DVT
Direct treatment with plasma exchange or IVIG