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90 Cards in this Set

  • Front
  • Back
What are infectious causes of Autonomic Neuropathy?
HIV
Chagas Disease
What are infectious causes of Cranial Neuropathy?
Lyme
VZV
What are infectious causes of distal symmetric polyneuropathy?
Lyme
Hepatitis C with Cryoglobulinemia
HIV
What are infectious causes of inflammatory demyelinating polyneuropathy?
Diphtheria
HIV
What are infectious causes of mononeuropathy multiplex?
CMV
HCV with cryoglobulinemia
HIV
Leprosy
Lyme
What are infectious causes of motor neuronopathy?
Poliomyelitis
West Nile Virus
What are infectious causes of radiculopathy?
CMV
HSV2
Lyme
Syphilis (Tabes Dorsalis involving sensory roots)
VZV
What is the most common neurologic complication of HIV and how does it present?

Exam findings?
Distal symmetric polyneuropathy

Numbness, paresthesia, hyperalgesia

Decrased distal vibratory and temperature sensation. Decreased or hyperalgesic pin prick
Preserved proprioception
Hyperactive reflexes sometimes from CNS HIV
What peripheral nerve problems occur with HIV?
Symmetric distal polyneuropathy
Demyelinating Polyneuropathies (CIDP occurs more commonly in HIV)
Mononeuropathy Multiplex
Autonomic Neuropathy
What two viral infections when occuring together are known to produce a significant mononeuropathy multiplex?
CMV and HIV
What is a varicella reactivation without rash called?
Zoster sine herpete
What can be done to diagnose shingles in a patient without a rash?
LP for VZV by PCR
What is therapy for shingles?
7-10 days of acyclovir, famciclovir, valacyclovir
Consider adding corticosteroids in patients withotu contraindications (improves motor outcomes)
What are the patterns of peripheral neuropathy that are associated with CMV?
Mononeuritis multiplex
Cauda equina syndrome
What are diagnostic studies that support a diagnosis of CMV associated neuropathy?
Nerve biopsy
CMV in other organs
CMV PCR positivity in CSF
What sort of peripheral neuropathy is associated with HSV infection?
HSV1 causes cranial neuropathy but only in context of meningitis or encephalitis

HSV2 causes radiculopathy involving lumbar and sacral nerve roots
What sort of peripheral neuropathy is associated with EBV infection?
Mononeuropathies and radiculopathies occur due to neurolymphomatosis in immunocompromised
What sort of peripheral nerve problems are associated with Hep C with cryoglobulinemia?
Sensorimotor polyneuropathy
Mononeuropathy
Mononeuropathy multiplex
What sort of peripheral nerve problems are associated with Hep B?
Very rare peripheral neuropathy. It basically does not occur.
What sort of peripheral nerve problems are associated with HTLV1?
Recent large cohort study showed no association
When do neurologic complications begin after primary infection in Diphtheria related disease?

Manifestations?

Treatment?
1 week -Paralysis of palate
2 weeks and 2 months - demyelinating polyneuropathy with limb and respiratory weakness

Antitoxin
Presentation of peripheral neuropathy associated with leprosy?
Multifocal neuropathy accompanied by macules and papules on trunk and abdomen in person from endemic area.

Enlarged nerves on exam with associated sensory and motor deficit
What are the peripheral nerve manifestations of Lyme?
Cranial neuropathies (facial neuropathy especially)
Radiculopathies

Less commonly brachial and lumbosacral radiculopathy and mononeuropathy multiplex

Even more rare are diffuse polyneuropathies similar to GBS
What are tests that can rule in or rule out neurosyphilils?
CSF VDRL is specific but not sensitive
CSF FTA is sensitive but not specific
Treatment of neurosyphilis?
IV penicillin x 10-14 days
What are the neurological manifestations of Chagas Disease?
Autonomic neuropathy leading mostly to GI symptoms such as megacolon and megaesophagus
Which HAART medications are most commonly associated with neuropathy?
Stavudine
Didanosine
Zalcitabine
Which nonHAART antibiotics produce neuropathy?
Dapsone
Isoniazid
Linezolid
Metronidazole

Chloramphenicol, chloroquine, fluroquinolones, griseofulvin, nitrofurantoin, podophyllin resin, sulfasalazine
Which toxic peripheral neuropathies are not reversible?
Sensory neuronopathies (involving the dorsal root ganglion
How can peripheral neuropathy from HIV be differentiated from peripheral neuropathy from HAART medications?
Check lactate - HAART meds causing neuropathy elevate lactate
What sort of neuropathy is caused by dapsone?
Non length dependent motor predminant neuropathy or mononeuropathy multiplex neuropathy with upper greater than lower weakness
Which medications used primarily in the treatment of cardiovascular disease have been known to cause neuropathy?

What sort of neuropathy do they cause?
Amiodarone
Procainamide
Hydralazine
Perhexiline
Statins - controversial

Amiodarone causes sensorimotor neuropathy often with severe motor symptoms
Procainamide causes a primarily demyelinating neuropathy
Hydralazine produces a sensory predominant axonal neuroapthy
How is hydralazine associated neuropathy treated?
pyridoxine
What are the major categories of chemotherapeutics which cause peripheral neuropathy and what do they include?
Mitotic spindle inhibitors - vinblastine, vincristine, paclitaxel, docetaxel

DNA binding compounds - cisplatin, carboplatin, oxaliplatin

Proteasome inhibitors - bortezomib is most common

Other cancer drugs - Suramin, thalidomide, misondazole
Which medications used for the treatment of rheumatologic disorders cause peripheral neuropathy?
Gold
Colchicine
Chloroquine
Tacrolimus
Leflunomide
How does acute pyridoxine toxicity present?

How does chronic pyridoxine toxicity present?
Severe sensory neuronopathy

diffuse paresthesia, proximal and distal sensory loss, autonomic dysfunction, sensory ataxia, reduced reflexes

Recovery from acute tox is poor
Recovery from chronic tox is good
Presentation of peripheral neuropathy from lead toxicity?

Associated symptoms?
Moderate to high level exposure: Axonal multifocal motor neuropathy with minor sensory symptoms. Asymmmetric finger and wrist drop resembling radial neuropathy

Chronic low level exposure: distal symmetric sensory loss

abdominal pain, constipation, anemia
Sources of arsenic toxicity?

Presentation of acute and chronic toxicity?
Contaminated water supply or exposure to byproducts of copper and lead smelting. Rarely deliberate poisoning

Axonal sensory and motor neuropathy

Acute and chronic length dependent neuropathy ccan occur

Acute toxicity causes nausea, vomiting, abdominal pain, diarrhea, psychosis, seizures

Chronic toxicity produces Mees lines, hyperpigmentation, palmar and solar keratoses
Treatment of arsenic toxicity?

Treatment of thallium toxicity?

Treatment of mercury toxicity?
Aggressive IV fluid replacement
Gastric lavage
Chelation (DMPS, DMSA)
Removal of exposing agent

Gastric lavage and Intragastric prussian blue or activated charcoal.
Chelating agents have no role
Some recommend hemodialysis

Gastric lavage
Activated Charcoal
Chelating agents
Removal of offending agent
Sources of thallium toxicity?

Presentation of acute and chronic thallium toxicity?
Intentional poisoning or accidental ingestion.

Acute toxicity - painful sensory neuropathy with preserved strength and reflexes, alopecia

Chronic exposure - axonal sensorimotor neuropathy
Sources of mercury poisoning?

Presentation of mercury poisoning?
Occupational exposure
Unclear if fish, old dental fillings, injections of thimerosal

Cognitive and behavioral changes, ataxia - sometimes neuropathy

Subacute exposure to vapor produces motor axonal neuroapthy

Chronic exposure produces painful axonal sensorimotor polyneuropathy
Presentation of ciguatera toxicity?
Gastroenteritis, perioral or distal paresthesias twelve to forty eight hours after ingestion. Paresthesias last for days to months.

Paradoxical disturbances in temperature sensation, myalgias, insomnia, prominent autonomic signs, sensory neuropathy

Death due to shock in 20%
Chronic syndrome with fatigability, depression, weakness in some
How to do patients affected by saxitoxin present?

How do patients affected by brevetoxin present?
Perioral tingling followed by limb/trunk paresthesias, weakness, incoordination and eventually respiratory failure
Recovery in 1 week with therapy

Gastroenteritis, paresthesia, dysesthesia, incoordination without weakness
Presentation of tetrodotoxin poisoning?
Perioral tingling in small amounts
Widespread paresthesia, weakness, autonomic failure, coma and death in larger amounts
Recovery in 4-5 days with supportive care
Presentation of organophosphate toxicity?

Prognosis?
Meiosis, muscle cramping, pulmonary edema, bradycardia, increased lacrimation and salivation

Neuropathy seven to ten days after exposure is length dependent sensorimotor axonal

Delayed myelopathy

Neuropathy slowly improves but myelopathy does not
Presentation of peripheral neuropathy from ethylene glycol toxicity?
Cranial and peripheral nerve dysfunction as early as five days and as late as twenty days following exposure

Severe axonal sensorimotor polyneuropathy
Source of hexacarbon toxicity?

Presentation?
Glue and solvent occupational exposure or recreational drug use

Early sensory symptoms followed by weakness and areflexia

Acute exposure - neuropathy is mixed demyelinating and axonal sensorimotor polyneuropathy
Chronic exposure leads to axonal sensory neuropathy
What are sources of acrylamide poisoning?

What are sources of Vacor poisoning?

What are sources of Buckthorn neuropathy?
Grouting agents, adhesives, flocculators

Rat poison

Buckthorn fruit (southwest US and Mexico)
How does neurotoxicity from nitrous oxide present?
Myeloneuropathy involving dorsal columns and large sensory nerve fibers, with preservation of spinothalamic spinal cord pathways.
What are the vitamin deficiencies most commonly associated with peripheral neuropathy?
B complex vitamins
Vitamin E
Copper
Niacin
What are causes of B6 deficiency?
Alcoholism
Isoniazid
Hydralazine
Penicillamine
Bariatric surgery
How does niacin deficiency present?

Which conditions predispose to niacin deficiency?
Pellagra

Skin lesions, diarrhea, depression, loss of appetite

Hartnup disease
Carcinoid syndrome
Alcoholism

No clear effect on peripheral nerves (usually concomminant B1 or B6 deficiency)
What conditions predispose to vitamin E deficiency?

Presentation?
Lipid malabsorption like abetalipoproteinemia, pancreatic enzyme deficiencies like cystic fibrosis
Bariatric surger

Cerebellar ataxia, sensory nerve abnormalities (sensory ataxia)
What conditions are associated with copper deficiency?
TPN
Zinc toxicity
Gastric bypass surgery

Menkes disease

Myelopathy, lower motor neuronopathy, optic neuropathy, axonal sensorimotor polyeuropathy
What are some nonmedical therapies for the treatment of painful small fiber neuropathy?
Acupuncture
Transcutaneous electrical stimulation devices
Decompression surgeries
Intradermal botulinum toxin type A
Topical capsaicin
Topical lidocaine
What are contraindications to cymbalta use?
severe hepatic dysfuncction
unstable arterial hypertension
What are the options for the treatment of peripheral nerve mediated neuropathic pain?
TCAs : Amitriptyline, Nortriptyline, Imipramine
SNRIs: Cymbalta, Venlafaxine
Alpha-2-delta ligand agonists: Gabapentin, pregabalin
Topical lidocaine, capsaicin patches, Tramadol, narcotics
How is capsaicin cream ideally initiated?
Several applications per day for several days before analgesic effect starts
What functions are served by large myelinated axons vs thinly myelinated axons vs small unmyelinated fibers?
Large myelinated axons - motor and sensory axons responsible for proprioception, vibration, light touch
Thinly myelinated axons - Seonsory fibers responsible for light touch, pain, temperature, preganglionic autonomic functions
Small unmyelinated fibers - pain and temperature, post ganglionic funcctions
What fine motor activities can be asked about in a patient with peripheral motor neuropathy?
Changes in handwriting, problems fastening jewelry, buttons or inserting or turning keys
What peripheral nerve disorders are known for anosmia?

Trigeminal sensory loss?
Refsum disease, B12 deficiency

Sjogren syndrome
Gait abnormalities in peripheral neuropathy?
Footdrop tends to result in steppage gait
Length dependent neuropathy causes more difficulty with heal walking than toe walking
Sensory ataxia is associated with wide based gait or difficulty with tandem
What are etiologies of acute onset neuropathies with presentation and progression over 1 month or less?
GBS, vasculitis, porphyria, infectious or toxic etiology.
What are etiologies of subacute onset neuropathy - over 6 months or less?
Toxic, nutritional deficiency, alignancy, paraneoplastic syndrome, metabolic abnormalities
What are etiologies of neuropathies with relapsing remitting course?
Demyelinating and subsequent remyelinating syndromes.

CIDP, porphyria, hereditary neuropathy with liability to pressure palsies, repeated toxic exposure, vasculitis
What are neuropathies with predominant motor involvement?
GBS, CIDP, MMN, porphyria, diptheria, lead intoxication, botulism, hereditary neuroapathies, toxic exposure related to dapsone, amiodarone, vincristine
What are causes of pure sensory neuropathy?
DM, Vit B12 deficiency, HIV, amyloidosis, leprosy, Sjogren syndrome, sarcoidosis, uremia, paraneoplastic syndromes, pyridoxine intoxication, hereditary neuropathies
What are causes of predominantly autonomic neuropathies?

What is ddx if acute or subacute?

What is ddx if chronic
diabetes mellitus, amyloidosis, GBS

Autoimmune autonomic ganglionopathy, paraneoplastic syndromes, GBS, botulism, toxic neuropathies, acute porphyria

Diabetes, amyloidosis, inherited diseases (i.e. HSAN), Fabry disease, Sjogren syndrome, toxic and infectious neuropathy
What are common causes of predominantly small fiber neuropathy?
DB, EtOH and other toxins, Fabry disease, Tangier disease, HIV, amyloidosis, Connective tissue disease, sarcoid, HSANs, idiopathic, medication
What are exam features suggestive of demyelinating neuropathy?
Weakness without atrophy
Length independent distribution with proximal predmoninant or asymmetric/patchy distribution, early involvment of proximal reflexes or myokymia
What is the ddx for demyelinating neuropathies?
Genetic (CMT Type 1, HNPP, Refsum, metachrommatic leukodystrophy)
CIDP
GBS
MMN
Paraproteinemia related neuropathiy
Infectious neuropathy (HIV, Lyme, Leprosy, Hepatitis C, Diptheria)
Toxin (N-hexane, amiodarone)
What pattern of weakness occurs in length dependent neuropathies?
Intrinsic foot muscles - toe flexor weakness and clawed toes.
Anterior tibial compartment weakness causing ankle dorsiflexion weakness with relatively preserved plantar flexion
Intrinsic hand muscle involvement after calf muscles are involved
Motor weakness greater in extensor than flexor group
Differential diagnosis for length independent neuropathy?
CIDP
MMN
Lyme disease
HIV
Sarcoidosis
Carcinoma
Amyloidosis
Lymphoma
Porphyria
Leprosy
Tangier disease
What does sensory neuropathy with patch distribution imply?
Sensory neuropathy directly affecting dorsal root ganglia
What is the differential for dorsal root gangionopathy?
Heavy metals
Paraneoplastic syndromes
Sjogren syndrome
HIV
Pyridoxine toxicity
Cisplatinum
Idiopathic
Which neuropathies involve the cranial nerves?
Celiac neuropathy - facial sensory symptoms
GBS - acute facial diplegia
CIDP - facial diplegia
Amyloidosis - facial diplegia
Lyme
Sarcoid
Melkersson Rosenthal syndrome - recurrent facial paralysis, lip and facial swelling, fissured tongue
What diagnostic testing is indicated in autonomic neuropathy of unknown etiology?
Evaluation for amyloidosis (fat pad biopsy, rectal biopsy, genetic testing)
Evaluation for porphyria, paraneoplastic disease, HSAN, Fabry disease
What features seperate the CMT I, CMT II, CMT IV, CMT X subtypes?
CMT I are AD demyelinating neuropathies
CMT II are AD axonal neuropathies
CMT IV are AR neuropathies
CMT X are X-linked neuropathies
What are the three phenotypes of CMT?
Classical phenotype - patients walk on time and develop weakness or sensory loss during first 2 decades - slow impairment but rarely require aides beyond ankle foot orthoses

Second phenotype - Infantile onset - do not walk until at least 15 months. Above the knee braces, walkers and wheelchairs required

Adult onset - Symptoms in adulthood, often not until after 40 years.
What is the most common viral infection associated with GBS?
CMV
What symptoms other than limb weakness are common with GBS?
Mild sensory loss
Pain especially in the lowe back, or aching of limbs
Areflexia early
Autonomic instability
Some with oculomotor paresis
What is the Miller Fischer triad?
Opthalmoplegia
Ataxia
Areflexia
What are MRI features of GBS?
Enhancement of spinal roots but this is nonspecific
What is the relapse rate for GBS?
2-6%
Typical presentation of Classical CIDP?
Symmetric proximal and distal weakness in both arms and legs
facial and extraocular weakness in only 10%
Symmetric numbness and tingling in hands and feet
Clinical features differentiating CIDP from GBS?
Nadir in GBS in 4 weeks without relapse
CIDP progresses over weeks and months
What are CIDP variant syndromes?
Multifocal motor neuropathy
Multifocal acquired demyelinating sensory and motor neuropathy (Lewis-Sumner syndrome)
Sensory CIDP
Pure Motor motor syndrome
How does Lewis-Sumner syndrome present?

What is its conventional name?
Asymmetric pattern of sensory and motor deficits

Mutifocal acquired demylinating sensory and motor neuropathy
Which paraprotein has the highest association with neuropathy?

What type of neuropathy is associated?
IgM
Less clear association between IgG and IgA

Most are demyelinating
Almsot half with IgM gammopathy have antibody directed against myelin-associated glycoprotein (MAG)
slowly progressive distal limb paresthesias, sensory loss, gait ataxia, tremor
Mild or absent weakness
What is used to treat anti-MAG neuropathy?
Rituximab
Poor response to corticosteroids, plasmapheresis, IVIG