Disorders of the PNS I (Classification, CMT & Diabetic Neuropathy) Flashcards
Which category of peripheral neuropathy is associated with motor and sensory loss in dermatomal distribution?
- Radiculopathy
- Often associated with disc & vertebral bone disease
RehabilitationConsiderations for diabetic neuropathies:
- Thorough assessment of sensory and motor systems
- Screen for autonomic neuropathy: specially cardiovascular AN: Squatting test: test for hydrostatic hypotension
- Safety assessment: at higher risk for falls
- Pain magament
Which category of peripheral neuropathy includes damaged to multiple peripheral nerves and is common in diabetes?
mononeuropathy multiplex
What is Acute Sensory Neuropathy?
- A type of diabetic neuropathy
- Rapid onset of severe burning pain, sharp “electric shock” hypersensitivity of feet
- Sympstons worsen at night
- Mild symmetric or no sensory loss but with allodynia
- Recovery possible within 1 year if stable blood glucose maintained
Prognosis of peripheral neuropathies:
- Depending on type of neuropathy recovery is usually slow.
- May have full recovery
- May have residual deficits
- With severe neuropathy, can develop chronic muscular atrophy, contractures, deformities
Motor & sensory changes resulting from PNS disease occur in:
- distribution of nerve roots
- plexi or peripheral nn themselves.
- Look at peripheral nerve distribution.
Which peripheral nerve disease involves mainly the peroneal nerve, affecting muscles in foot & lower leg but can progress to nereves of forearms & hands.
Charco Marie Tooth Disease AKA Hereditary Motor and Sensory Disease AKA peroneal muscular atrophy
The PNS includes motor, sensory and autonomic nerves:
- Cranial nn
- Spinal nn
- Spinal nerve roots
- Peripheral nn
- Autonomic system
Which category of peripheral neuropathy includes damage to spinal roots?
- radiculopathy
- dermatomal distribution
In which type of diabetic neuropathy the patient shows a rapid onset of: severe burning pain, deep aching pain, sudden sharp “electric shock” sensation, and hypersensitivity of feet?
Acute Sensory Neuropathy
AUTONOMIC Signs & Symptoms of Neuropathic Dysfunction include:
- Vasomotor disturbances (orthostatic hypotension)
- Alterations in sweating
- Trophic changes of skin & nails
Medical Management of diabetic neuropathies:
- Good metabolic control: control of blood sugar levels
- Symptomatic management
- Medication for painful sensory neuropathies
- Prevention of complications
MOTOR Signs & Symptoms of Neuropathic Dysfunction include:
- Weakness (usually distal weakness first)
- Decreased DTRs
- Atrophy
- Cramping with fatigue
- Hypotonicity or flaccidity
Etiology of Charcot Marie-Tooth Disease:
- Inherited as autosomal dominant, autosomal recessive or X-linked pattern
- Various types: CMT1 most common autosomal dominant pattern.
which type of diabetic neuropathy presents with the characterictic “stocking - glove” sensory loss?
chronic sensorimotor neuropathy or diabetic polyneuropathy
Etiology/Pathogenesis of Diabetic Neuropathy:
- Metabolic impairments related to hyperglycemia
- Vascular changes
- Reduced nerve growth factor (NGF)
categories of peripheral neuropathy:
1. Symmetric polyneuropathy: stocking and glove
2. Mononeuropathy: mononeuropathy multiplex is common in diabetes
3. Plexopathy: injury to brachial, lumbar, or sacral plexus.
4. Radiculopathy: injury to spinal roots, dermatomal distribution
Diagnosis of CMT:
- History,
- Clinical exam,
- genetic studies,
- electrophysiologic studies (NCV/EMG),
- nerve biopsy
Pathology and two types of Charcot Marie-Tooth Diseas:
Gene mutations result in abnormal structure &/or function of the peripheral nerves
- CMT1: Segmental demyelination of fibular (peroneal) nerve
- CMT2: Associated with axonal degeneration -Onset varies between 2nd and 7th decades
What is the incidence of Charcot-Marie-Tooth Disease?
Onset?
- 1 in 2500 persons in the USA
- Onset in childhood or adulthood: Most often in adolescence or early adulthood
what is chronic sensorymotor neuropathy?
- AKA diabetic polyneuropathy
- Most common
- loss of position sense & vibration: large fibers
- loss of pain & temp: small finbers
- dull. aching pain in limbs and burning sensations especially at night
Autonomic diabetic neuropathy is manifested where?
In multiple systems:
- Cardiavascular: tachycardia, exercise intolerance, orthostatic hypotension, dizziness.
- GI: esophagus dysfunction, diarrhea, constipation
- Genitourinary: incontinence, erectile dysfunction
- Other: sweating, heat intolerance, dry skin, blurred vision
SENSORY Signs & Symptoms of Neuropathic Dysfunction:
- Anesthesia
- Hipothesia
- Hyperesthesia
- Impaired position sense and vibration: Large fiber problem
- Impaired pinprick & temperature: Small fiber problem
Which is the most common hereditary disorder of peripheral nerves:
Charcot Marie-Tooth Disease AKA hereditary motor and sensory neuropathy (HSMD) or peroneal muscular atrophy
Which category of peripheral neuropathy includes injury to nerves in brachial, lumbar or sacral plexus?
plexopathy
Clinical Manifestations of CMT:
- Distal symmetric muscle weakness: dorsiflexors and evertors: Footdrop (steppage) gait.
- Atrophy
- Decreased deep tendon reflexes
- Skeletal deformities: pes cavus, hammer toes
- Loss of proprioception in feet and amkle
- Decrease cutaneous sensation in feet and lower legs.
- With progression of the disease, distal UEs become invloved:
- Weakness & wasting of hand intrinsics
- Wasting of forearm mm
- Rarely, respiratory muscle weakness in later stages
What are the goals of physicial therapy in treating a patient with CMT:
Minimize deformity and Maximize function
Which category of peripheral neuropathy includes dysesthesia and decreased sensation?
Symmetric polyneuropathy
Which category of peripheral neuropathy results from compressive lesion of a single nerve? Ex. Carpal tunnel syndrome
Mononeuropathy
Forms of neuropathies associated with diabetes:
- Polyneuropathy
- Mononeuropathy
- Plexopathy
- autonomic neuropathy
Which is the most common diabetic neuropathy?
Chronic Sensorimotor neuropathy or AKAdiabetic polyneuropathy
what is the pathology of peripheral neuropathy?
involves damage to myelin, axons, or both
Which neuropathy can occur with poorly controoled DM?
Hyperglycemic Neuropathy
How may petients with diabetes develop some type of neuropathy?
50%-60%
Focal neuropathies:
Type of diabetic neuropathy:
- Cranial nerve focal nerupathies: oculomotor nerve most commonly affected
- Limb focal neuropathies: median (not to be confused with carpal tunnel syndrome), ulnar and peroneal nerve most commonly affected.
what are trophic changes?
- Autonomic neuropathic dysfunction; changes resulting from interruption of nerve supply.
- Skin and nails
Also known as hereditary motor and sensory neuropathy (HMSN) or peroneal muscular atrophy
Charcot Marie Tooth Disease
Treatment of CMT:
- No specific treatment
- Symptomatic interventions to help maintain function.
- Orthotics; skin care precautions
What is hyperglycemic neuropathy?
- Rapidily reversible
- Occurs with poorly controlled DM
- Distal symmetric sensory changes: burning, paresthesias, tenderness in legs & feet
- Symptoms resolve when blood sugar becomes controlled
Classification of diabetic neuropathies:
1.
Interventions for CMT:
- Streteching
- ROM
- Orthostics
- Gait training
- Skin care education
Prognosis of CMT
- Slowly progressive disease
- Weakness & contractures lead to gait abnormalities, falls, and difficulty with writing and manipulating objects with hands
Pain Management (rahabilitation considerations) of Painful Diabetic Neuropathy: what works and what doesn work?
- “electrical stimulation is probably effective in lessening the pain of PDN and improving QOL”
- “electromagnetic field treatment, low-intensity laser treatment, and Reiki therapy are probably not effective for the treatment of PDN.”
- Exercise
- Functional treatment
Course/ Prognosis of Diabetic Neuropathies:
- Slowly progressive
- Some improve; some plateau
- Autonomic involvement associated with increased mortality risk
What is Wallerian degeneration?
- dying back of axon distal to lesion,
- occurs in axonal degeneration
Peripheral Neuropathy Etiology (causes)
- Systemic/metabolic diseases: Diabetes, hypothyroidism, renal failure, AIDS, Lyme disease, RA
- Vitamin deficiency
- Exogenous/environmental toxins
-
Hereditary neuropathies:
- Charcot-Marie-Tooth disease
-
Mechanical pressure/trauma
- Compression
- Entrapment
