Chapter 27: Diseases of Peripheral Nerves Flashcards
Why is the peripheral nerve dysfunction caused by neuronopathies equally likely to affect proximal and distal parts of the body?
Damage at the level of neuronal cell body
Which structures are injured in the CMT2 variant of CMT disease and when does it present?
Axonal injury; typically severe w/ early childhood onset
Which type of neuropathy is seen with lepromatous leprosy (Hansen Disease) and which sensory fibers will be lost?
- Symmetric polyneuropathy affected cool distal extremities and face
- Involves pain fibers, loss of sensation = injury; since pt’s are rendered unaware of injurious stimuli –> large traumatic ulcers
If VZV is reactivated it travels along sensory nerves and leads to what?
Painful, vesicular skin eruption (shingles) in a sensory dermatomal distribution
Morphologically what is the most prominent lesion seen in Guillain-Barre Syndrome?
Segmental demyelination affecting peripheral nerves
Upon sural nerve biopsy in patient with Chronic inflammatory Demyelinating Poly(radiculo)neuropathy what is a characteristic finding?
Onion-bulbs: excessive proliferation –> multiple layers of Schwann cells wrap around an axon like the layers of an onion
Demyelinating forms of Charcot-Marie-Tooth (CMT) disease are associated with what morphological features?
- Demyelination and remyelination including Schwann cell hyperplasia —> Onion-bulb formation
- Hyperplasia may be so severe that involved nerve is palpably enlarged
Which type of neuropathy is the most common paraneoplastic form and which malignancy is it most commonly associated with?
Sensorimotor neuronopathy in setting of Small cell lung cancer = most common
Which nerve is affected in Morton neuroma and what histologic finding is seen?
- Interdigital nerve at intermetatarsal sites –> foot pain; “walking on a marble”
- Histologically = perineural fibrosis
What are the primary targets of damage in demyelinating neuropathies?
Schwann cells w/ their myelin sheaths
Clinical presentation of Guillain-Barre Syndrome is dominated by what signs/sx’s; what characteristic CSF finding will there be?
- Ascending paralysis and areflexia
- DTR’s lost early in the process
- ↑ CSF protein levels w/ little or no CSF pleocytosis (inflammatory cells remain confined to the roots
How is Chronic inflammatory Demyelinating Poly(radiculo)neuropathy treated?
Glucocorticoids + IVIg + plasmapheresis
Which dermatomes are commonly affected by VZV reactivation; what kind of damage is seen?
- Thoracic or trigeminal nerve dermatomes
- Neuronal destruction and loss of affected ganglia
- Axonal degeneration of periphral nerves after death of sensory neurons
What is a a distinctive presentation of neuropathy associated with monoclonal gammopathies?
POEMS: Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes
In lepromatous leprosy (Hansen Disease) Schwann cells are invaded by Mycobacterium leprae and what pattern of peripheral nerve damage will be seen?
Segmental demyelination and remyelination + loss of both myelinated and unmyelinated axons
Familial amyloid polyneuropathies are mainly due to germline mutations in which gene?
Transthyretin gene
Focal destruction of which neuronal structures may be seen with reactivation of VZV?
Large motor neurons in anterior horn or cranial nerve nuclei
“Saturday night palsy” is due to compression of which nerve?
Radial nerve in the upper arm
Prevalence of peripheral neuropathy associated with diabetes is dependent on what?
Duration of the disease
2/3’s of Guillain-Barre Syndrome cases are preceded by what; which etiologies have been implicated?
- Acute, influenza-like illness
- Campylobacter, CMV, EBV, and Mycoplasma penumoniae, or prior vaccination
Other than peripheral neuropathy, what is another manifestation of diabetic nervous system dysfunction that is often seen?
Autonomic dysfunction: postural hypotension, incomplete bladder emptying (↑ infections) and sexual dysfunction
Mononeuritis multiplex describes a disease process that damages several nerves in a haphazard fashion and is commonly due to what?
Vasculitis i.e., polyarteritis nodosum
Which painful nodule composed of non-neoplastic haphazard whorled proliferation of axonal processes and assoc. Schwann cells results from failure of axons to find their distal target during regeneration ?
Traumatic neuroma aka “pseudotumor”
Characteristic “stocking and glove” distribution of sensory deficits is seen with what anatomic pattern of peripheral neuropathies?
Polyneuropathies
Which 2 features of Chronic inflammatory Demyelinating Poly(radiculo)neuropathy distinguish it from Guillain-Barre?
Time course (presence at least 2 months) and response to steroids
What is the electrophysiologic hallmark of axonal neuropathies vs. demyelinating neuropathies?
- Axonal neuropthay = a reduction in signal strength
- Demyelinating neuropathy = slowed nerve conduction velocity
Chronic inflammatory Demyelinating Poly(radiculo)neuropathy is characterized by what type of neuropathy?
Symmetrical mixed sensorimotor polyneuropathy that persists for 2 months or more
What are “positive sx’s” associated with diabetic peripheral neuropathy?
Paresthesias and dyesthesias = painful sensations
Diabetic peripheral neuropathy is characterized by a relative loss of which size and type of nerve fibers?
Small myelinated and unmyelinated fibers
How is Guillain-Barre Syndrome managed clinically?
Plasmapheresis and IV Ig
More localized nerve involvement associated with granulomatous nodules in the dermis is characteristic of what form of leprosy?
Tuberculoid leprosy = TH1 (cell-mediated) response
Uremic neuropathy seen in setting of renal failure is a distal, symmetric neuropathy often associated w/ what signs and sx’s?
Muscle cramps + distal dysesthesias + ↓ DTRs
What is the most common acquired inflammatory peripheral neuropathy?
Chronic inflammatory Demyelinating Poly(radiculo)neuropathy
Histologic features of Guillain-Barre Syndrome are characterized by what?
Inflammation** and **demyelination of spinal nerve roots and peripheral nerves = radiculoneuropathy
Peripheral nerve dysfunction as a result of Diptheria is due to what; what are the early sx’s and later findings?
- Result of diptheria exotoxin
- Early loss of proprioception and vibratory sensation
- Acute peripheral neuropathy assoc. w/ prominent bulbar and respiratory m. dysf.
What is the most common pattern of peripheral neuropathy seen with Diabetes?
Ascending distal symmetric sensorimotor polyneuropathy
What is the most common subtype of hereditary motor and sensory neuropathy (CMT) disease, what is the inheritance pattern, when and how does it present?
- CMT1 = group of autosomal dominant disorders
- Presents in 2nd decade w/ slowly progressive distal demyelinating motor and sensory neuropathy
Biopsies of the affected peripheral nerves/arterioles in diabetes will show what finding and with what stain?
Endoneurial arterioles show thickening, hyalinization, and intense PAS-(+) of their walls + extensive reduplication of basement membranes
