Acute peripheral neuropathy

Locked-in Syndrome
The states of coma and the vegetative state must be distinguished from a syndrome in which
there is little or no disturbance of consciousness, but only an inability of the patient to respond adequately.
The latter is referred to as the locked-in syndrome or the deefferented state.
The locked-in syndrome is most often caused by a lesion of the ventral pons (basis pontis) as a result of occlusion
of the basilar artery. Such an infarction spares both the somatosensory pathways, and the ascending neuronal
systems responsible for arousal and wakefulness, as well as certain midbrain elements that allow the eyelids to be
raised in wakefulness; the lesion essentially interrupts the corticobulbar and corticospinal pathways, depriving the
patient of speech and the capacity to respond in any way except by vertical gaze and blinking.
Severe motor neuropathy (e.g., Guillain-Barre syndrome), pontine myelinolysis, or periodic paralysis may have a
similar effect.

SYNDROME OF ACUTE MOTOR PA RALYS IS
WITH VA RIABLE DISTURBANCE OF
SENSORY AND AUTONOMIC FUNCTION
A number of differences separate the polyneuropathies
in this category:
(1) acute inflammatory demyelinating or axonal polyneuropathy (GBS)
(2) vasculitic polyneuropathies
(3) porphyria
(4) certain toxic polyneuropathies
(5) acute sensory and autonomic polyneuropathies
Of these various acute polyneuropathic diseases, the
Guillain-Barre demyelinative syndrome, because of its
frequency and gravity, is most demanding of the physi
cian's attention

CASE
• A 15 yr old male,came to ED with complaints of pain in the calf of
the right leg on Tuesday(1week ago),followed by weakness of the
both lower limbs the next day morning.was bed ridden after the
onset of weakness.his bowel and bladder are intact.he is able to
feel sensation of clothes over the lower limbs.he felt heaviness of
the limbs..taken to local hospital was given treatment..where he
noticed weakness in the upper limbs also.
• no symptoms of cranial nerve involvement.
• no hx fever at the onset of weakness.
• no hx recent immunization or no h/o dog bite.
• no hx seizures episodes.
• no hx similar complaints in the past.
• no hx similar complaints in the family.

Clinical examination
• Patient was consciuos coherent
• no pallor ,icterus,clubbing,lymphadenopathy,edema.
• His BP is 110/70 mm Hg in supine,100/70 mm Hg on standing.no postural
hypotension.
• Pulse -82/min regular,all peripheral pulses felt.
• Respiratory rate -18 /min.breath holding time –able to count upto 45 in single
breath.

NERVOUS SYSTEM
• Higher intellectual functions intact
• Cranial nerves normal
• Motor system showed hypotonia of all four limbs.
• Grade 3/5 in upperlimbs,2/5 across the hip joint,knee joint,o/5 power across
ankle joint.
• Hyporeflexia all DTRs,absent ankle
• Babinski negative,abdominal present
• Sensory intact
• Gait –stance …
• Other systems normal.

Key points
• Rapid progressive ascending paralysis.
• Acute onset
• Motor paralysis
• Intact sensory
• No cranial nerve involvement
• No bladder involvement
• No fever at onset of weakness
• Diagnosis………………??????????????????

OTHER NAMES
• LANDRY’S ASCENDING PARALYSIS
• ACUTE INFLAMMATORY DEMYELINATING POLYNEUROPATHY (AIDP)
• ACUTE IDIOPATHIC POLYRADICULONEURITIS
• ACUTE IDIOPATHIC POLYNEURITIS
• FRENCH POLIO
• LANDRY GUILLAIN BARRE SYNDROME

INTRODUCTION
Acute,frequently severe and
fulminant polyradiculoneuropathy
that is autoimmune in nature.

Antecedent events (causes)
• 70% cases –post infectious .
• 1-3 weeks after an acute infectious process respiratory or GIT.
• 20-30% cases –campylobacter jejuni
• Other agents –HHV (EBV,CMV)
• Mycoplasma pneumoniae
• Recent immunisation –swine influenza vaccine,older rabies vaccine
(nervous system)
• Can be seen in patients with lymphoma,HIV positive,SLE.

AIDP
• Adults > children
• Rapid recovery
• Anti GM1 ab (50%)
• Demyelinating
• First attack on schwann cell surface
• Widespread myelin damage
• Lymphocyte infiltration
• Variable sec axonal damage

AMAN (ACUTE MOTOR AXONAL
NEUROPATHY)
Acute motor axonal neuropathy (AMAN) is a variant of Guillain-Barré syndrome. It is characterized by
acute paralysis and loss of reflexes without sensory loss.
The syndrome typically presents as a progressive flaccid symmetric paralysis with areflexia, often causing
respiratory failure
• Children ,young adults
• Seasonal
• Recovery rapid
• Anti GD1 a ab
• Axonal
• First attack on motor nodes of ranvier
• Macrophage activation
• Axonal damage is variable

MILLER FISCHER VARIANT
• Adults,children
• Uncommon
• Anti GQ 1 b anitbodies >90%
(Not seen in other forms of GBS
Unless there is EOM invovlement)
• Pupillary paralysis

Clinical manifestations
FEVER AND CONSTITUTIONAL SYMPTOMS ARE ABSENT AT THE ONSET
MOTOR SYSTEM :
• Rapidly evolving areflexic motor paralysis with or without sensory disturbance.
• Ascending type of paralysis
• RUBBERY LEGS.
• Weakness evolves over hours –days.
• Legs>arms
DEEPTENDON REFLEXES:
Reflexes attenuate,disappear in few days of onset.

CRANIAL NERVES:
Facial diparesis seen in 50% affected individuals.
Lower cranial nerves affected –bulbar weakness – difficulty in handling
secretions,maintaining airway.
Ophthalmoplegia –miller fischer variant
Pupillary paralysis
Optic atrophy

SENSORY SYSTEM:
Largely myelinated fibres are severely affected.
Proprioception is more affected than pain temperature sensation.
BLADDER:
Only in severe cases,transiently.
If bladder dysfunction is a prominent feature and comes early in the course,think
other than GBS – spinal cord disease.

• PAIN:
• Deep aching pain may be present the previous day in
weakened muscles.
• Initially at onset –neck,shoulder,back,diffusely over spine -50%
cases.
• Dysesthetic pain in extremities
• Self limited usually
• Responds to analgesics

• AUTONOMIC INVOLVEMENT:
• Common
• Seen even in mild cases
• Wide fluctuation in blood pressure
• Postural hypotension
• Cardiac dysrryhthmias
• Close monitoring and management
• Can be fatal.
• All require hospitalisation
• 30% require ventilatory support

course
• Pattern of rapidly progressive
(evolving) paralysis with areflexia
• Usually does not progress beyond four weeks after reaching a
plateau.

Lab features
CSF :
• Raised CSF protein 1-10 g/l (100-1000mg/dl)
• Without accompanied by pleocytosis
• Usually normal in <1week
• Increased proteins at the end of first week
• 10-100/ul elevation
• Think of HIV ,CMV in case of pleocytosis.
ELECTRODIAGNOSTIC TESTS:
May be normal
Lags behind clinical events
demyelination - prolonged distal latencies,conduction velocity slowing,condcution block.
Reduced amplitude of compound action potential without conduction slowing

Criteria for diagnosis
REQUIRED :
• 1.progressive weakness of 2 or more limbs due to neuropathy.
• 2.areflexia
• 3.disease course < 4 weeks
• 4 exclusion of other causes (vasculitis,PAN,SLE,churg strauss
syndrome,toxins,lead,botulism,diptheria,porphyria.,cauda equinal
syndrome)
SUPPORTIVE:
• 1.relatively symmetric weakness
• 2.mild sensory involvement
• 3.facial nerve or other cranial nerve involvement
• 4.absence of fever
• 5.typical CSF profile(aceelualr,increase in protein level)
• 6.electrophysiologic evidence of demyelination

treatment
• Initiate as soon as possible.
• Each day counts
• 2 weeks after the first motor symptoms – immunotherapy is no longer effective.
• IVIg
• Plasmapheresis
• Combination is not effective
• IVIg-first choice,easy to administer
• Five daily infusions 2g/kg body weight
• Plasmapheresis 40-50ml/kg four times a week
• Treatment reduces need for ventilation by half.,increases full recovery at an year.
• Glucocorticoids are not effective in GBS.
• Conservative management in mild cases.

 This type of polyneuropathy appears after several days or more of bacterial sepsis or other
overwhelming infection (now called systemic inflammatory response syndrome
[SIRS]) and multiple organ failure, and is preceded in most instances by a confusional state or a
depressed state of consciousness ("septic encephalopathy").
 The neuropathic process, predominantly of motor type, varies in severity from an
electrophysiological abnormality without overt clinical signs, to quadriparesis with respiratory
failure.
 Sensory symptoms and signs are variable but tend to be mild. Usually the cranial nerves are
spared and there are few or no dysautonomic manifestations.
In general,
 The EMG and NCS findings of a primary axonal process with early denervation and a normal
CSF distinguish this entity from the typical demyelinative form of GBS.
Critical Illness Polyneuropathy

Local action of the exotoxin may paralyze pharyngeal and laryngeal
muscles (dysphagia, nasal voice) within 1 or 2 weeks after the onset
ofthe infection and shortly thereafter may cause blurring of vision
because of paralysis of accommodation, but these and other cranial
nerve symptoms may be overlooked. At this stage, the cranial
neuropathy must be distinguished from that of GBS, botulism, and most
of all, from myasthenia gravis.
A polyneuropathy, appearing 5 to 8 weeks later, takes the form of an
acute or subacute limb weakness with paresthesias and distal loss of
vibratory and position sense.
The weakness characteristically involves all extremities at the same time
or may descend from arms to legs. The patient may be unable to stand
or walk and occasionally the paralysis is so extensive as to impair
respiration. The CSF protein is usually elevated (50 to 200 mg/dL) .
The important pathologic change is one of segmental demyelination
without inflammatory reaction of spinal roots, sensory ganglia, and
adjacent spinal nerves.Anterior hom cells, axons, peripheral nerves
Diphthertic Polyneuropathy

• Treatment
Diphtheria antitoxin, given within 48 h of the onset of the infection,
Antitoxin is probably of little value once the polyneuropathy
begins.Thereafter, treatment is purely symptomatic, along the lines
indicated for GBS.

Acute porphyria
Acute porphyrias include forms of the
disease that typically cause nervous
system symptoms which appear quickly
and can be life threatening.
Acute porphyria attacks are rare be for
puberty and after menopause in women.
symptom may last one to two weeks and
usually improve slowly after the attack.

Possible sign and symptoms:
severe abdominal pain
swelling of the abdomen (abdominal distention)
pain in your chest,legs or back
constipation or diarrhea
vomiting
insomnia
 heart beat you can feel (palpitation)
high blood pressure
anxiety or restlessness
seizures
mental change ,such as confusion,hallucination,disorientation or paranoia
breathing problems
muscle pain ,tingling,numbness,weakness or paralysis
red or brown urine

stopping medications that may have triggered symptoms
medication to control pain,nausea and vomiting
prompt treatment of infections or other illness that may have
caused symptoms
intravenous sugar(glucose) or sugar taken by mouth,if able
maintain an adequate intake of carbohydrates
intravenous fluids to combat dehydration
injection of hemin,a medication that is a form of heme , to
limit the body's production of porphyrin

the peripheral nerves may be affected by a wide variety of toxins
including metals, drugs, organophosphates, and industrial solvents.
As a rule, the neuropathies induced by these agents fall into the
subacute and chronic categories.
However, certain drugs-notably
triorthocresyl phosphate (TOCP) and other organophosphates ,
thallium
and rarely, arsenic
produce a polyneuropathy that may be fatal in a matter of days.
Acute Toxic Polyneuropathies

Severe and permanent motor paralysis is caused by TOCP; this ultimately proves to be a result of involvement
of both upper and lower motor neurons.
Some cases of arsenical and possibly mercurial polyneuropathy may also develop acutely.

Thallium salts
when taken in sufficient amount, produce a clinical picture resembling that of GBS or an acute
sensory polyneuropathy.
If the salts are taken orally,there is first abdominal pain, vomiting, and diarrhea, followed within a
few days by pain and tingling in the toes and fingertips and then rapid weakening of muscles of
the limbs, initially the distal ones.
As the weakness progresses, the tendon reflexes diminish.
Pain sensation is reduced more than tactile, vibratory; and position sense.
Persistent acral pain with allodynia has been a major feature in 3 of the 5 patients we have
examined; in 2 of our patients there was no weakness, only sensory loss and ataxia.
All cranial nerves except the first and eighth may be affected;
facial palsies, ophthalmoplegia, nystagmus, optic neuritis with visual impairment, and vocal cord
palsies are additional abnormalities but only in the most severely affected patients.
The CSF protein rises to more than 100 mg. Death may occur in the first 10 days as a result of
cardiac arrest.
The early onset of painful paresthesias, sensory loss, and pain localized to joints, back, and chest,
as well as rapid loss of hair (after a week or two), all serve to differentiate this neuropathy from
GBS,porphyria, and other acute polyneuropathies.
Thallium salts act like potassium and a high intake of potassium chloride hastens the excretion of
thallium. Chelating agents are of unproven value but are usually included in treatment.

On occasion, a vasculitic polyneuropathy as an isolated process or
associated with lupus erythematosus, polyarteritis nodosa, and related
disorders may develop as rapidly as GBS. Three of our patients with
polyarteritis and one with Churg-Strauss disease became completely
paralyzed within a week and one died of intestinal perforation.
We have observed a few patients with alcoholism,occult carcinoma,
Hodgkin disease, and renal transplantation develop an acute
polyneuropathy, as rapid in its evolution as GBS, and acute episodes of
this type have also been described in patients with Refsum disease.
Other Acute Polyneuropathies

Acute peripheral neuropathy

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