ppt on peripheral neuropathy.pptx

Approach to Peripheral
Neuropathy
- Dr Anurag Ghotkar

• Peripheral neuropathy can involve sensory motor or autonomic
nerves .
• They can affect any part of the nerve -

Classification of peripheral nerve diseases
• Mononeuropathy
• Plexopathy
• Radiculopathy
• Mononeuritis multiplex
• polyradiculoneuropathy

Features of CNS
localisation
Brisk DTR , Increased tone , pyraminal
tract pattern ( one sided upper and
lower limb affected , flexors of lower
and extensors of upper limb affected )
Features of Spinal
cord Localisation
Sensory loss in both legs with a
sensory level on trunk
One side pin prick loss and other side
proprioception + weakness

Approach to patient
• 3 Main Goals
• Site of lesion – by history examination and Edx.
• Cause of lesion- 7 key questions .
• Treatment .

7 key questions to find out the cause
• System affected ?
• Distribution of weakness
• Nature of sensory loss
• UMN findings
• Temporal evolution
• Hereditary ?
• Significant medical history

Severe sensory ataxia
• Sensory gangionopathy
• Sjogren syndrome OR Paraneoplastic neuropathy

• Sensory neuropathy affecting large fibres can be further

7 key questions to find out the cause
• System affected ?
• Distribution of weakness
• Nature of sensory loss
• UMN findings
• Temporal evolution
• Hereditary ?
• Significant medical history - Alcohol , DM , surgery , history of
diarrhoea , diet history, use of dentures- zinc can cause copper
deficiency , excess use of vit B6 , SLE , toxins .

Distribution of weakness
• Ask 2 questions about the weakness
• Is it proximal with distal weakness or only distal weakness.
• Is it symmetrical or asymmetrical/focal weakness .

Pattern 4 : Asymmetric proximal.
and distal weakness with.
sensory loss
-polyradiculopathy
Plexopathy
Meningeal carcinomatosis
Sarcoidosis
Amyloidosis
Herediatry plexopathy
idiopathic

Polyneuropathy with
1)Facial nerve involvement – GBS>CIDP , sarcoidosis,
Lyme , HIV , Tangier , Gelsolin FAP.
2)Upper limb involvement – MMN , MADSAM , Lead
, porphyria ,Tangier , FAP2 , Hereditary motor
neuropathy.
3)predominantly motor – AMAN/GBS/CIDP , MMN
,Lead , CMT , Diabetic lumbar radiculoplexopathy,
Neuropathy with osteosclerotic myeloma .

• In case of symmetrical pattern – send CBC , LFT , KFT , electrolytes ,
HbA1c , TFT , B12 , folic acid , ESR , RA , ANA , SPEP ,FLC ratio , urine
for bence jones proteins.

Edx
• NCS
• Needle EMG
• Autonomic function testing .

• Painful sensory neuropathy – advice : FBS/PPBS/HbA1c ,

• Weakness of finger extensor or wrist
• Anemia
• Basophilic stippling of RBC

GBS
• CSF proteins – elevated
• CSF pleocytosis –if present look for secondary causes – HIV,
Sarcoidosis , lymes disease , leukemia infiltrating nerve roots .
• Abnormal LFT in GBS – check HHH, CMV , EBV .
• Porphyria screening for GBS – If Axonal GBS or GBS with abdo. pain
/psychiatric illness/autonomic dysfunction .

CIDP
• Symmetric proximal and distal weakness , length dependant loss of
large fibre sensation and areflexia >8weeks
• No ANS involvement
• No respiratory involvement
• Adv- screen for monoclonal gammopathy
• Hallmark- sural sparing .
• If Ig G4 Ab to neurofascin and contactin 2 – have sensory ataxia ,
tremor , distal weakness . Responds to rituximab .

Role of Biopsy
• Punch biopsy from skin
• Sural nerve biopsy.
• Superficial peroneal nerve with peroneus brevis muscle(combination
Biopsy)

•Mononeuritis multiplex – non contiguous involvement
of 2 or more peripheral nerves

Most common hereditary neuropathy - CMT
• Type 3- dejerine sotta syndrome – infant or child with demyelinating
sensory motor neuropathy – NCV – 5 to 10 m/sec .
• Type1 vs Type 2 CMT .
CMT1 CMT2
Child Adult
NCV in Arm :- 20 to 25 m/sec >38m/sec
Duplication of PMP22-
TYPE 1A
Mpzero mutation – Type
1b CMT
Mitofuscin 2 gene in outer
membrane of
mitochondria
C/F – Foot drop
O/E – sensation loss , DTR loss , Atrophy of anterior compartment of knee (Inverted
champagne bottle legs ) , pes cavus

HNPP
• Del of one PMP22 gene .
• History of trivial compression of nerves like leaning on elbow
• Pressure
• Mononeuropathy
• Numbness /painless and weakness

HNA
• Recurrent pain , weakness , sensory loss
• Brachial plexus affected .
• hypotelorism, epicanthal folds, cleft palate, syndactyly,
micrognathia, and facial asymmetry, are evident in
some individuals
• EDx - axonal process.
• mutations in septin 9 (SEPT9)-role in cell division.

HSAN
• sensory and autonomic dysfunction >> muscle weakness
• There are no medical therapies available .
• prevention and treatment of mutilating skin and bone lesions
• only HSAN1 typically presents in adults. autosomal dominant
• second through fourth decades of life.
• degeneration of small myelinated and unmyelinated nerve fibers
• leading to severe loss of pain and temperature sensation, deep dermal ulcerations,
recurrent osteomyelitis, Charcot joints, bone loss, gross foot and hand deformities, and
amputated digits.
• do not complain of numbness, they often describe burning, aching, or lancinating pains.
• Autonomic neuropathy is not a prominent feature, but bladder dysfunction and reduced
sweating in the feet may occur.
• HSAN1A - mutations in serine palmitoyltransferase long-chain base 1 (SPTLC1) gene.

FABRY’S DISEASE
X-linked dominant
• men > women
• Angiokeratomas - reddish-purple maculopapular
lesions around the umbilicus, scrotum, inguinal
region, and perineum
• Burning or lancinating pain in the hands and feet
• premature atherosclerosis lead to death by the fifth
decade of life.
• dilated cardiomyopathy.
• mutations in the α-galactosidase gene  ceramide
trihexoside in nerves and blood vessels.
• Enzyme replacement therapy with α-galactosidase B

ADRENOLEUKODYSTROPHY/
ADRENOMYELONEUROPATHY
X-linked dominant
peroxisomal transmembrane adenosine triphosphate-binding cassette (ABC) transporter gene.
1)CNS abnormalities
2)third to fifth decade - mild to moderate
peripheral neuropathy combined with
progressive spastic paraplegia (30 %cases)
3)Rare- adult-onset spinocerebellar ataxia with adrenal insufficiency.
Edx - axonopathy with secondary demyelination.
Nerve biopsies - a loss of myelinated and unmyelinated nerve fibers with lamellar inclusions in the cytoplasm
of Schwann cells.
Very-long-chain fatty acid (VLCFA) levels (C24, C25, and C26) are increased in the urine.
Diets low in VLCFAs and supplemented with Lorenzo’s oil (erucic and oleic acids) reduce the levels of VLCFAs
and increase the levels of C22 in serum, fibroblasts, and liver.

REFSUM’S DISEASE
• Classic tetrad of
• (1) peripheral neuropathy,
• (2) retinitis pigmentosa,
• (3) cerebellar ataxia
• (4) elevated CSF protein concentration.
• Serum phytanic acid levels are elevated.
• Sensory and motor NCS reveal reduced amplitudes, prolonged latencies, and slowed
conduction velocities
• Nerve biopsy demonstrates a loss of myelinated nerve fibers, with remaining axons often
thinly myelinated and associated with onion bulb formation.
phytanoylCoA α-hydroxylase (PAHX).>> peroxin 7 receptor protein (PRX7)
• Treatment is removal of phytanic precursors (phytols: fish oils, dairy products, and
ruminant fats) from the diet.

TANGIER DISEASE
autosomal recessive
(1) asymmetric multiple mononeuropathies
(2) slowly progressive symmetric polyneuropathy
predominantly in the legs, or
(3) pseudo-syringomyelia pattern with dissociated
sensory loss (i.e., abnormal pain/temperature
perception but preserved position/ vibration in the
arms.
(4) tonsils - swollen and yellowish-orange
(5) splenomegaly and lymphadenopathy.
(6) ABC1 gene mutated -> reduced HDL and increased
triacylglycerol
(7) Nerve biopsies- reveal axonal degeneration with
demyelination and remyelination.
(8) accumulation of lipid in Schwann cells of small
myelinated nerves.

Porphyria associated with peripheral neuropathy
• Acute attack of porphyria = sharp abdominal painagitation, hallucinations, or seizuresSeveral days later, back and extremity
pain  weakness ensues, mimicking GBS
• Weakness can involve the arms or the legs and can be asymmetric, proximal, or distal in distribution, as well as affecting the face
and bulbar musculature.
• Dysautonomia and signs of sympathetic overactivity are common (e.g., pupillary dilation, tachycardia, and hypertension).
• Constipation, urinary retention, and incontinence can also be seen.
• CSF protein is typically normal or mildly elevated.
• SIADH hyponatremia in some .
• Acute intermittent porphyria (AIP)
• hereditary coproporphyria (HCP)
• variegate porphyria (VP).
• The urine may appear brownish in color secondary to the high concentration of porphyrin metabolites. Accumulation of
intermediary precursors of heme
• Edx -marked reductions in CMAP amplitudes and active axonal degeneration on needle EMG.
• Treatment with Intravenous glucose is started at a rate of 10–20 g/h.
• If there is no improvement within 24 h
• intravenous hematin 2–5 mg/kg per day for 3–14 days should be administered.

FAMILIAL AMYLOID POLYNEUROPATHY

Hypothyroidism
proximal myopathy >> neuropathy, most typically CTS >> Rarely, a
generalized sensory polyneuropathy characterized by painful
paresthesias and numbness in both the legs and hands can occur.

PRIMARY OR AL AMYLOIDOSIS
Due to plasmacytomas
• 30% - polyneuropathy, most typically painful dysesthesias and burning
sensations in the feet.
• Trunk can be involved --mononeuropathy multiplex pattern.
• CTS occurs in 25% of patients .
• slowly progressive weakness develops along with large-fiber sensory
loss. Most patients develop autonomic involvement
• protein is often increased (with normal cell count), and thus, the
neuropathy may be mistaken for CIDP .
• Nerve biopsy-- axonal degeneration and amyloid deposition in either a
globular or diffuse pattern
• Chemotherapy with melphalan, prednisone, and colchicine and
autologous stem cell transplantation may prolong survival.

DIABETIC NEUROPATHY -most common cause of
peripheral neuropathy in developed country.
Risk factors
• long-standing poorly controlled DM
• presence of retinopathy and nephropathy.

• DSPN is most common
• sensory loss in toes
• Legs
• fingers and arms .
• trunk (chest and abdomen),
initially in the midline
anteriorly and later
extending laterally.
• Tingling, burning, deep
aching pains
• NCS-- reduced amplitudes
and mild to moderate
slowing of conduction
velocities
• Nerve biopsy– axonal
degeneration

Diabetic Autonomic Neuropathy
• abnormal sweating
• dysfunctional thermoregulation
• dry eyes and mouth
• pupillary abnormalities
• cardiac arrhythmias
• postural hypotension
• GI abnormalities (e.g., gastroparesis,
postprandial bloating, chronic diarrhea,
or constipation)
• genitourinary dysfunction (e.g.,
impotence, retrograde ejaculation,
incontinence)
• Tests of autonomic function are
generally abnormal, including
sympathetic skin responses and
quantitative sudomotor axon reflex

Diabetic Radiculoplexus
Neuropathy (Diabetic
Amyotrophy or Bruns-Garland
Syndrome)
• Neuropathy is often accompanied or heralded weight loss.
• severe pain in the low back, hip, and thigh in one leg.
Rarely, the diabetic polyradiculoneuropathy begins in both
legs at the same time .
• Atrophy and weakness of proximal and distal muscles in
the affected leg become apparent within a few days or
weeks.
• Weakness usually progresses over several weeks or months
but can continue to progress for 18 months or more.
• CSF protein elevated, cell count normal.
• ESR increased.
axonal degeneration along
with perivascular
inflammation.
severe pain treated with -
glucocorticoids.

Diabetic Mononeuropathies or Multiple
Mononeuropathies
• most common-- median neuropathy at the wrist
ulnar neuropathy at the elbow
peroneal neuropathy at the fibular head
sciatic, lateral femoral, cutaneous, or cranial neuropathies also occur .
• seventh nerve palsies are relatively common .
• In diabetics, a third nerve palsy is most common, followed by sixth
nerve and, less frequently, fourth nerve palsies.
• Diabetic third nerve palsies are characteristically pupil-sparing.

Sjogren Syndrome
• Most common  length-dependent axonal
sensorimotor neuropathy characterized
mainly by sensory loss in the distal extremities
• Trigeminal nerve
• Sensory ganglionopathies -progressive
numbness and tingling of the limbs, trunk,
and face in a non-length-dependent manner
such that symptoms can involve the face or
arms more than the legs. Severe vibratory and
proprioceptive loss leading to sensory ataxia.
Occasionally responds to IvIg.
• NCS-reduced amplitudes of sensory studies in
the affected limbs.
• Nerve biopsy-axonal degeneration.
• rarely --necrotizing vasculitis.
• When vasculitis is suspected,
immunosuppressive agents may be beneficial.

Rheumatoid Arthritis
• 50% of patients
• If Vasculitic
neuropathymononeuropathy
multiplex or generalized symmetric
pattern
• If Drug induced e.g., tumor necrosis
blockers, leflunomide
Nerve biopsy - fibrinoid necrosis of
vessel walls.
Immunomodulating therapies.

SLE
• Small fibre neuropathy-
slowly progressive sensory
loss beginning in the feet.
burning pain and
paresthesias with normal
reflexes
• Less common- multiple
mononeuropathies?
secondary to necrotizing
vasculitis
Rx immunosuppressive
• Rarely- GBS .

SYSTEMIC SCLEROSIS (SCLERODERMA)
• distal symmetric, mainly sensory
polyneuropathy
• trigeminal nerve, producing numbness
and dysesthesias in the face.
• Multiple mononeuropathies
• axonal sensory greater than motor
polyneuropathy.
• MCTD- mild distal axonal sensorimotor
polyneuropathy in ~10% .

Sarcoidosis
• seventh nerve, which can be affected
bilaterally.
• radiculopathy or polyradiculopathy
• GBS or CIDP.
• multiple mononeuropathies or
• generalized, slowly progressive, sensory
greater than motor polyneuropathy
• small-fiber neuropathy
• Edx- axonal neuropathy.
• Nerve biopsy - noncaseating granulomas
infiltrating the endoneurium, perineurium, or
epineurium
• lymphocytic necrotizing angiitis.
• Rx Glucocorticoids or immunosuppressive
agents.

CELIAC DISEASE
• A generalized sensorimotor polyneuropathy, pure motor neuropathy,
multiple mononeuropathies, autonomic neuropathy, small-fiber
neuropathy, and neuromyotonia have all been reported in association
with celiac disease or antigliadin/antiendomysial antibodies
• Nerve biopsy may reveal a loss of large myelinated fibers.
• may be secondary to malabsorption
Hypereosinophilic syndrome -
• A generalized peripheral neuropathy or a mononeuropathy multiplex
occurs in 6–14% of patients

Ulcerative colitis and Crohn’s disease
GBS, CIDP, generalized axonal sensory
or sensorimotor polyneuropathy,
small-fiber neuropathy, or
mononeuropathy
Cause-
autoimmune
nutritional (e.g., vitamin B12
deficiency)
treatment related (e.g.,
metronidazole, TNFalfa)

Uremic neuropathy
• length-dependent numbness, tingling,
allodynia, and mild distal weakness
• GBS can occur rarely –and improves with
renal dialysis or transplantation
• Mononeuropathies - CTS.
• Ischemic monomelic neuropathy can
complicate arteriovenous shunts created in
the arm for dialysis
• Edx - length-dependent, primarily axonal,
sensorimotor polyneuropathy.
• Sural nerve biopsies
• RRT

CLD
• generalized sensorimotor
neuropathy characterized by
numbness, tingling, and minor
weakness in the distal aspects
• sensory > motor axonopathy.
• Sural nerve biopsyboth
segmental demyelination and
axonal loss.

Weakness developing in critically ill patients
while in the ICU
• Muscle stretch reflexes are absent or reduced
• elevated serum CK would point to CIM as opposed to CIP.
• NCS reveal absent or markedly reduced amplitudes of motor and sensory studies in CIP, whereas sensory studies are relatively preserved in
CIM.
• Needle EMG usually reveals profuse positive sharp waves and fibrillation potentials, and it is not unusual in patients with severe weakness to
be unable to recruit motor unit action potentials.
• axonal degeneration.

LEPROSY
• Neuropathies are most common in borderline .
• Superficial cutaneous nerves of the ears and distal limbs are commonly affected.
• Mononeuropathies, multiple mononeuropathies, or a slowly progressive symmetric
sensorimotor polyneuropathy may develop
• Sensory NCS - absent in the lower limb and are reduced in amplitude in the arms.
• Motor NCS - reduced amplitudes in affected nerves
• occasionally can reveal demyelinating features.

LYME DISEASE
• Borrelia burgdorferi transmitted by the deer tick Ixodes dammini
• Facial neuropathy is most common and is bilateral in about half of
cases, which is rare for idiopathic Bell’s palsy. Involvement of nerves is
frequently asymmetric.
• polyradiculoneuropathy or multiple mononeuropathies
• axonal degeneration with perivascular inflammation.

Diphtheria
• flulike - generalized myalgias, headache, fatigue, low-grade fever, and
irritability within a week to 10 days of the exposure
• 20–70% develop diffuse axonal sensorimotor polyneuropathy by toxin .
• 3-4 weeks after infectiondecreased sensation in their throat, dysphagia,
dysarthria, hoarseness, and blurred vision
• 2-3months later- generalized polyneuropathy --numbness, paresthesias,
and weakness of the arms and legs and occasionally ventilatory failure
• CSF protein elevated with or without lymphocytic pleocytosis.
• Antitoxin and antibiotics should be given within 48 h of symptom onset
• Early treatment reduces the cardiomyopathy
but not alter the natural history of the associated peripheral neuropathy.
The neuropathy usually resolves after several months.

HIV
(1) distal symmetric polyneuropathy (DSP) – most common
Numbness and painful paresthesias involving the distal extremities
(2) AIDP -usually develops at the time of seroconversion
CIDP - can occur any time
(3) multiple mononeuropathies (e.g., vasculitis, CMV- related)
(4) Acute progressive lumbosacral polyradiculoneuropathy – CMV related – neutrophilic
pleocytosis with low csf sugars .
(5) autonomic neuropathy
(6) sensory ganglionitis/ganglionopathy – sensory ataxia .

HVZ reactivation
• Two-thirds - severe pain and paresthesias in dermatomal region followed
within a week or two by a vesicular rash in the same distribution .
• Weakness in muscles innervated by roots corresponding to the
dermatomal distribution of skin lesions occurs in 5–30% of patients.
• Approximately 25% of affected patients have continued pain (postherpetic
neuralgia [PHN]).
EBV
- GBS, cranial neuropathies, mononeuropathy multiplex, brachial plexopathy,
lumbosacral radiculoplexopathy, and sensory neuronopathies

Malignancy
■PARANEOPLASTIC SENSORY NEURONOPATHY/ GANGLIONOPATHY –
small-cell lung carcinoma
Asymmetric numbness and paresthesias in the distal extremities
loss of proprioception sensory ataxia
Weakness due to - myelitis, motor neuronopathy, or concurrent LEMS.
confusion, memory loss, depression, hallucinations or seizures, or cerebellar ataxia. Polyclonal
antineuronal antibodies (IgG)against Hu antigen.
CSF - normal or mild lymphocytic pleocytosis and elevated protein.
■TUMOR INFILTRATION –
mononeuropathy, mononeuropathy multiplex, polyradiculopathy, plexopathy, or even a generalized
symmetric distal or proximal and distal polyneuropathy .
often painful .
may improve with- treatment of leukemia or with glucocorticoid.

Bone marrow transplant
Neuropathies because of -
• chemotherapy
• Radiation
• Infection
• autoimmune
• GVHD may develop cranial neuropathies, sensorimotor
polyneuropathies, multiple mononeuropathies, and severe generalized
peripheral neuropathies resembling AIDP or CIDP
may improve by increasing immunosuppressive therapy and resolution of
the GVHD.

MULTIPLE MYELOMA –
most common pattern - distal, axonal, sensory, or sensorimotor
polyneuropathy > chronic demyelinating polyradiculoneuropathy .
amyloid polyneuropathy- painful paresthesias, loss of pinprick and
temperature discrimination, and autonomic dysfunction (suggestive of
a small-fiber neuropathy) and CTS.
Expanding plasmacytomas can compress cranial nerves and spinal roots
Chemotherapeutic agents usually cause a sensory greater than motor
length-dependent axonal neuropathy or neuronopathy/
ganglionopathy

TOXIC NEUROPATHIES
■ Amiodarone neuromyopathy -if medication for 2–3 years. segmental
demyelination and axonal loss. Electron microscopy reveals lamellar or
dense inclusions in Schwann cells, pericytes, and endothelial cells.
■Colchicine - proximal weakness and numbness and tingling in the
distal extremities. vacuolar myopathy and axonal degeneration.
■ Thalidomide - numbness, painful tingling, and burning discomfort in
the feet and hands and less commonly muscle weakness and
atrophy.loss of large-diameter myelinated fibers and axonal
degeneration and Degeneration of dorsal root ganglion cells

• B6 toxicity at high doses (116 mg/d) - Severe sensory neuropathy
with dysesthesias and sensory ataxia.
• NCS -reduced SNAP amplitudes with relatively preserved CMAPs.
• Nerve biopsy -,axonal loss of fiber at all diameters
• Standard doses of INH (3–5 mg/kg per day) are associated with a 2%
incidence of neuropathy,
• whereas neuropathy develops in at least 17% of patients taking in
excess of 6 mg/kg per d.
• The elderly, malnourished, and “slow acetylators” are at
increasedincreasingly
• Prophylactic administration of pyridoxine 100 mg/d can prevent the
neuropathy from developing

ANTIRETROVIRAL AGENTS
• The nucleoside analogues and nucleoside reverse
transcriptase inhibitor (NRTI) are used to treat HIV
infection.
• major dose- predominantly sensory, length-
dependent, symmetrically painful neuropathy
• Zalcitabine (ddC) at at doses >0.18 mg/kg per d, it is
associated with a subacute onset of severe burning
and lancinating pains in the feet and hands
HEXACARBONS (n-HEXANE, METHYL n-BUTYL
KETONE)/GLUE SNIFFER’S NEUROPATHY
• water-insoluble industrial organic solvents
present in some glues.
• Glue sniffing or through skin absorption--
profound subacute sensory and motor
polyneuropathy.
• NCS -decreased amplitudes of the SNAPs and
CMAPs with slightly slow CVs.
axonal degeneration
MERCURY
• paresthesias in hands and feet
• progress proximally and may involve the face and
tongue.
• Motor weakness
• axonal sensorimotor polyneuropathy

Lead
• ingest lead-based paints in older buildings and in industrial
workers exposed to lead-containing products.
• Encephalopathy
• insidious and progressive onset of weakness usually beginning in
the arms, in particular involving the wrist and finger extensors,
resembling a radial neuropathy
• Sensation is generally preserved
• autonomic nervous system can be affected
• microcytic hypochromic anemia with basophilic stippling of
erythrocytes, an elevated serum lead level, and an elevated
serum coproporphyrin level.
• A 24-h urine collection demonstrates elevated levels of lead
excretion.
• NCS - reduced CMAP amplitudes, while the SNAPs are typically
normal.
• Rx Chelation therapy EDTA , BAL, and penicillamine.

THALLIUM - a rodenticide.
burning paresthesias of the feet, abdominal pain, and vomiting. Increased thirst, sleep
disturbances, and psychotic behavior may be noted.
first week - pigmentation of the hair, an acne-like rash in the malar area of the face, and
hyperreflexia.
second and third week - autonomic instability
Hyporeflexia and alopecia - third or fourth week following exposure.
With severe intoxication, proximal weakness and involvement of the cranial nerves can occur.
Some patients require mechanical ventilation due to respiratory muscle involvement.
lethal dose - 8 to 15 mg/kg body weight. Death can result in <48 h
NCS- axonal sensorimotor polyneuropathy.
acute intoxication potassium ferric ferrocyanide to stop gi absorption.
diuresis is essential to help eliminate thallium

ARSENIC
• neuropathy manifests 5–10 days after ingestion of arsenic
and progresses for several weeks, sometimes mimicking
GBS.
• abrupt onset of abdominal discomfort, nausea, vomiting,
pain, and diarrhea
• neuropathy after 5 to 10 daya - burning pain in the feet
and hands. loss of the superficial epidermal layer 
patchy regions of increased or decreased pigmentation
• Can progress like GBS .
Mee’s lines - chronic exposure , can also be seen following
thallium poisoning.
• Anemia with stippling of erythrocytes
• aplastic anemia
• Increased CSF protein levels without pleocytosis can be
seen; this can lead to misdiagnosis as GBS.
• NCS - axonal sensorimotor polyneuropathy; however,
demyelinating features can be present.

Nutritional neuropathies
COBALAMIN (VITAMIN B12 )- numb hands typically appear before lower extremity paresthesias
A preferential large-fiber sensory loss affecting proprioception and vibration with sparing of small-fiber
modalities is present; an unsteady gait reflects sensory ataxia.
Diffuse hyperreflexia and absent Achilles reflexes Optic atrophy and, in severe cases, behavioral
changes ranging from mild irritability and forgetfulness to severe dementia and frank psychosis may
appear.
CNS manifestations, especially pyramidal tract signs, may be missing, and in fact, some patients may
only exhibit symptoms of peripheral neuropathy
Edx shows an axonal sensorimotor neuropathy. CNS involvement produces abnormal somatosensory
and visual evoked potential latencies.
Serum methylmalonic acid and homocysteine, the metabolites that accumulate when cobalamin-
dependent reactions are blocked, are elevated.
Antibodies to intrinsic factor are present in ~60% and antiparietal cell antibodies in ~90% of individuals
with pernicious anemia.
1000 μg cyanocobalamin IM weekly for 1 month and monthly thereafter. Or
An oral cobalamin dose of 1000 μg/d should be sufficient.
50% of patients exhibit some permanent neurologic deficit.

THIAMINE DEFICIENCY
• Cause- chronic alcohol abuse, recurrent vomiting, total parenteral
nutrition, and bariatric surgery , inappropriately restrictive diets.
• greatest sources are unrefined cereal grains, wheat germ, yeast, soybean
flour, and pork.
• Dry beriberi -neuropathic symptoms.
• wet beriberi –cardiac manifestations (edema)
• mild sensory loss and/or burning dysesthesias in the toes and feet and
aching and cramping in the lower legs. Pain may be the predominant
symptom.
• With progression - nonspecific generalized polyneuropathy, with distal
sensory loss in the feet and hands.
• Erythrocyte transketolase activity .
• Edx shows nonspecific findings of an axonal sensorimotor polyneuropathy.
• intravenously or intramuscularly at a dose of 100 mg/d.

Vitamin E deficiency
Cause –
lipid malabsorption or disorders of
vitamin E transport.
Abetalipoproteinemia- pigmentary
retinopathy, acanthocytosis, and
progressive ataxia.
cystic fibrosis and steatorrhea.
cholestatic and hepatobiliary
disorders
short-bowel syndromes
c/f – insidious onset and gradually progressive
spinocerebellar ataxia and polyneuropathy, thus
resembling Friedreich’s ataxia or other
spinocerebellar ataxias
posterior column dysfunction - impaired joint
position and vibratory sensation.
polyneuropathy there is hyporeflexia,
plantar responses may be extensor as a result of
the spinal cord involvement
ophthalmoplegia, pigmented retinopathy, night
blindness, dysarthria, pseudoathetosis,
dystonia, and tremor
Diagnosis is made by measuring α-tocopherol
levels in the serum
Edx shows features of an axonal neuropathy.
treatment 1500–6000 IU/d in divided doses.

COPPER DEFICIENCY – myeloneuropathy
• lower limb paresthesias, weakness, spasticity, and gait difficulties
• Large-fiber sensory function is impaired, reflexes are brisk, and plantar responses are extensor.
• In some cases, light touch and pinprick sensation are affected,
• NCS indicate sensorimotor axonal polyneuropathy in addition to myelopathy
• Zinc upregulates enterocyte production of metallothionine, which results in decreased absorption of
copper.
oral copper sulfate or gluconate 2 mg one to three times a day.
VITAMIN B6 DEFICIENCY – patients on isoniazid or
hydralazine.
generalized axonal sensorimotor polyneuropathy
50–100 mg/d pyridoxine .
PELLAGRA (NIACIN DEFICIENCY)
peripheral neuropathy resembles beriberi.
Treatment is with niacin 40–250 mg/d.

Weight loss surgeries
several months after surgery
numbness and paresthesias in the feet
Management –
parenteral vitamin/ thiamine,
parenteral nutritional support
reversal of the surgical bypass.

MONONEUROPATHIES
• 1)MEDIAN NEUROPATHY CTS - a compression of median nerve in the carpal
tunnel at the wrist.
• numbness and paresthesias variably in the thumb, index, middle, and half of
the ring finger.
• At times, the paresthesias can include the entire hand and extend into the
forearm or upper arm or can be isolated to one or two fingers.
• The signs of CTS are decreased sensation in the median nerve distribution;
• reproduction of the sensation of tingling when a percussion hammer is tapped
over the wrist (Tinel sign) or the wrist is flexed for 30–60 s (Phalen sign); and
• weakness of thumb opposition and abduction.
• Edx -slowing of sensory and, to a lesser extent, motor median potentials across
the wrist.
• Ultrasound can show focal swelling of the median nerve at the wrist.
• Treatment options consist of avoidance of precipitating activities;
• control of underlying systemic-associated conditions if present;
• NSAID
• neutral (volar) position wrist splints, especially for night use;
• glucocorticoid/anesthetic injection into the carpal tunnel; and
• surgical decompression by dividing the transverse carpal ligament.

2)ULNAR NEUROPATHY AT THE ELBOW—
“CUBITAL TUNNEL SYNDROME”
• paresthesias, tingling, and numbness in the medial
hand and half of the fourth and the entire fifth
fingers, pain at the elbow or forearm, and
weakness.
• Signs consist of decreased sensation in an ulnar
distribution
• Tinel’s sign at the elbow, and weakness and atrophy
of ulnar-innervated hand muscles.
• Froment sign indicates thumb adductor weakness
and consists of flexion of the thumb at the
interphalangeal joint when attempting to oppose
the thumb against the lateral border of the second
digit
• Edx may show slowing of ulnar motor NCV across
the elbow with prolonged ulnar sensory latencies.
• Ultrasound can show swelling of the ulnar nerve
around the elbow
• Treatment consists of avoiding aggravating factors,
using elbow pads, and surgery to decompress the
nerve in the cubital tunnel.

3)RADIAL NEUROPATHY
• wrist drop; finger extension weakness;
thumb abduction weakness; and sensory loss
in the dorsal web between the thumb and
index finger. Triceps and brachioradialis
strength is often normal, and triceps reflex is
often intact.
• Most cases of radial neuropathy are
transient compressive (neuropraxic) injuries
that recover spontaneously in 6–8 weeks. If
there has been prolonged compression and
severe axonal damage, it may take several
months to recover.
Treatment consists of cock-up wrist and finger
splints, avoiding further compression, and
physical therapy to avoid flexion contracture.
If there is no improvement in 2–3 weeks, an
Edx study is recommended to confirm the
clinical diagnosis and determine the degree of

4)LATERAL FEMORAL CUTANEOUS NEUROPATHY
(MERALGIA PARESTHETICA)
• paresthesias, numbness, and occasionally pain in
the lateral thigh.
• Symptoms are increased by standing or walking
and are relieved by sitting. There is normal
strength, and knee reflexes are intact.
• Edx is only needed to rule out lumbar plexopathy,
radiculopathy, or femoral neuropathy. If the
symptoms and signs are classic, EMG is not
necessary.
• Symptoms often resolve spontaneously over weeks
or months, but the patient may be left with
permanent numbness.
• Treatment consists of weight loss and avoiding
tight belts.
• Analgesics in the form of a lidocaine patch,
nonsteroidal agents, and occasionally medications
for neuropathic pain can be used Rarely, locally
injecting the nerve with an anesthetic can be tried.
• There is no role for surgery.

5)Femoral neuropathies
• can arise as complications of retroperitoneal
hematoma, lithotomy positioning, hip
arthroplasty or dislocation, iliac artery
occlusion, femoral arterial procedures,
infiltration by hematogenous malignancy,
penetrating groin trauma, pelvic surgery
including hysterectomy and renal
transplantation, and diabetes (a partial form
of lumbosacral diabetic plexopathy); some
cases are idiopathic.
• difficulty extending their knee and flexing the
hip.
• Sensory symptoms occurring either on the
anterior thigh and/or medial leg occur in only
half of reported cases.
• The quadriceps (patellar) reflex is diminished.

6)SCIATIC NEUROPATHY
• hip arthroplasty, pelvic procedures ,
prolonged lithotomy position, trauma,
hematomas, tumor infiltration, and vasculitis
• Weakness may involve all motions of the
ankles and toes as well as flexion of the leg
at the knee;
• abduction and extension of the thigh at the
hip are spared.
• Sensory loss occurs in the entire foot and the
distal lateral leg
• The ankle jerk and, on occasion, the internal
hamstring reflex are diminished or more
typically absent on the affected side.
• The peroneal subdivision of the sciatic nerve
is typically involved disproportionately to the
tibial counterpart. Thus, patients may have
only ankle dorsiflexion and eversion
weakness with sparing of knee flexion, ankle
inversion, and plantar flexion; these features
can lead to misdiagnosis of a common
peroneal neuropathy.
7)PERONEAL NEUROPATHY
• foot drop (ankle dorsiflexion, toe extension, and
ankle eversion weakness) and variable sensory
loss, which may involve the superficial and deep
peroneal pattern. There is usually no pain.
• Onset may be on awakening in the morning.
Peroneal neuropathy needs to be distinguished
from L5 radiculopathy
• In L5 radiculopathy, ankle invertors and evertors
are weak and needle EMG reveals denervation.
Edx can help localize the lesion. Peroneal motor
conduction velocity shows slowing and
amplitude drop across the fibular head
• Management consists of rapid weight loss and
avoiding leg crossing.
• Foot drop is treated with an ankle brace.
• A knee pad can be worn over the lateral knee to
avoid further compression.
• Most cases spontaneously resolve over weeks or
months.

Radiculopathies are most often due to
compression from degenerative joint
disease and herniated disks, but there are
a number of unusual etiologies

Plexopathy
1)BRACHIAL PLEXUS
• Immune-Mediated Brachial Plexus
Neuropathy (IBPN)= acute brachial plexitis
= neuralgic amyotrophy = Parsonage-
Turner syndrome.
• acute onset of severe pain in the shoulder
region. The intense pain usually lasts
several days to a few weeks, but a dull
ache can persist. Individuals who are
affected may not appreciate weakness of
the arm early in the course because the
pain limits movement. However, as the
pain dissipates, weakness and often
sensory loss are appreciated.
• The most common pattern of IBPN
involves the upper trunk
• Edx is useful to confirm and localize the
site(s) of involvement.
• Empirical treatment of severe pain with
glucocorticoids .
• Associated with Neoplasms - may be
• Primary brachial plexus tumors (are less common )- schwannomas,
neurinomas, and neurofibromas.
• Secondary tumors/malignant-are more common - may arise from local
tumors, expanding into the plexus. For example, a Pancoast tumor of
the upper lobe of the lung may invade or compress the lower trunk,
whereas a primary lymphoma arising from the cervical or axillary
lymph nodes may also infiltrate the plexus. Pancoast tumors typically
present as an insidious onset of pain in the upper arm, sensory
disturbance in the medial aspect of the forearm and hand, and
weakness and atrophy of the intrinsic hand muscles along with an
ipsilateral Horner’s syndrome. Chest computed tomography (CT) scans
or magnetic resonance imaging (MRI) can demonstrate extension of
the tumor into the plexus
• Metastatic –from breast cancer into the axillary lymph nodes and local
spread into the nearby nerves.
• Perioperative Plexopathies (Median Sternotomy) The most common
surgical procedures associated with brachial plexopathy as a
complication are those that involve median sternotomies (e.g.,
openheart surgeries and thoracotomies). Brachial plexopathies occur
in as many as 5% of patients following a median sternotomy and
typically affect the lower trunk. Thus, individuals manifest with
sensory disturbance affecting the medial aspect of forearm and hand
along with weakness of the intrinsic hand muscles. The mechanism is
related to the stretch of the lower trunk, so most individuals who are
affected recover within a few month

LUMBOSACRAL PLEXOPATHIES
• Plexopathies are typically recognized when motor, sensory, and if applicable, reflex deficits occur
in multiple nerve and segmental distributions confined to one extremity. If localization within the
lumbosacral plexus can be accomplished, designation as a lumbar plexopathy, a sacral plexopathy,
a lumbosacral trunk lesion, or a panplexopathy is the best localization that can be expected.
• Although lumbar plexopathies may be bilateral, usually occurring in a stepwise and
chronologically dissociated manner, sacral plexopathies are more likely to behave in this manner
due to their closer anatomic proximity.
• The differential diagnosis of plexopathy includes disorders of the conus medullaris and cauda
equina (polyradiculopathy)
• If there is a paucity of pain and sensory involvement, motor neuron disease should be considered
as well.
• Diabetic radiculopathy common cause of painful leg weakness.
• Other causes - retroperitoneal hemorrhage , carcinoma of the cervix, endometrium, and ovary;
osteosarcoma; testicular cancer; MM; lymphoma; acute myelogenous leukemia; colon cancer;
squamous cell carcinoma of the rectum; adenocarcinoma of unknown origin; and intraneural
spread of prostate cancer.

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