Transcript MOTOR NEURON DISEASE

Peripheral nerve disease
Peripheral neuropathy

inherited and acquired pathological
processes may affect the nerve roots
(radiculopathy), the nerve plexuses
(plexopathy) and/or the individual nerves
(neuropathy).
anatomical pattern ---Single root ----monoradiculopathy
Mononeuropathy means focal involvement of a single nerve and implies
a local process. Direct trauma, compression or entrapment, vascular
lesions, and neoplastic infiltration diabetes mellitus,
hypothyroidism,acromegaly, alcoholism, and HNPP
Multiple mononeuropathy or mononeuropathy multiplex
signifies simultaneous or sequential damage to multiple noncontiguous
nerves
vasculitis or microangiopathy in diabetes mellitus, infectious,
granulomatous,
leukemic, or neoplastic infiltration, sarcoidosis and -Multifocal motor
neuropathy
Polyneuropathy is characterized by symmetrical, distal motor, and
sensory deficits that have a graded increase in severity distally and by
distal attenuation of reflexes. The sensory deficits produce a stockingglove pattern.
Classification
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Acute
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Subacute
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Classification
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Small nerve fibers
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Large nerve fibers
Chronic
Classification
• Axonal
• Demylination
• Mixed
predominantly motor manifestations
Multifocal motor neuropathy
Guillain-Barre syndrome
Acute motor axonal neuropathy
Porphyric neuropathy
Chronic inflammatory polyradiculoneuropathy
Neuropathy with osteosclerotic myeloma
Hereditary motor sensory neuropathies[ Charcot-MarieTooth disease)
Lead intoxication
Predominant sensory involvement
diabetes; carcinoma; Sjogren's syndrome;
dysproteinemia; acquired immunodeficiency
syndrome (AIDS); vitamin B12 deficiency;
celiac disease; intoxications with cisplatin,
thalidomide, or pyridoxine; and inherited
idiopathic sensory neuropathies.
Autonomic dysfunction is seen in GBS
amyloid and diabetic, idiopathic panautonomic
neuropathy, Porphyria, Hereditary sensory and
autonomic neuropathy
Clinical features
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Motor nerve involvement produces features
of a lower motor neuron lesion weakness
fasciculation cramps
sensory nerve : Parasthesia numbness
analgesia anasthesia
Autonomic fibre involvement may cause
postural hypotension due to disruption of
vasomotor control, sweating, cardiac rhythm,
and gastrointestinal, bladder and sexual
functions
Acute Inflammatory Demyelinating Polyradiculoneuropathy (Guillain-Barre Syndrome)
motor weakness, areflexia, paresthesias with slight sensory loss, and increased
protein in CSF without pleoeytosis (albuminocytological dissociation). The
frequent finding of motor conduction block and reduced NCVs provided
electrophysiological
confirmation of widespread demyelination
Features required for diagnosis
Progressive weakness of both legs and arms
A reflexia
Clinical features supportive of diagnosis
Progression 1 day -4 weeks
Relative symmetry of signs
Mild sensory symptoms or signs
Cranial nerve involvement (bifacial palsies)
Recovery beginning 2—4 wks after progression ceases
Autonomic dysfunction
Absence of fever at onset
Laboratory features supportive of diagnosis
Elevated cerebrospinal fluid protein with normal cells count
Elcctrodiagnostic features of nerve conduction slowing or block
Clinical features
1-4 weeks after respiratory infection or diarrhoea (particularly
Campylobacter) in 70% of Patients.
Distal paraesthesia and numbness (often severe) precede a
rapidly ascending muscle weakness, from lower to upper
limbs, more marked proximally than distally.
Facial
and bulbar weakness commonly develops,
Clinical features
respiratory weakness requiring ventilatory support
occurs in 20% of cases. In most patients, weakness progresses
for 1-4 weeks, but rapid deterioration to respiratory failure can
develop within hours. On examination there is diffuse weakness
with widespread loss of reflexes.
GBS Types
•Acute inflammatory demyelinaring polyradiculoneuropathy
•Acute motor axonal neuropathy
•Acute motor sensory axonal neuropathy
•Millar-Fisher syndrome (o p h t h a l m o p l e g i a , a t a x i a a n d a r e f l e x i a )
•Acute pandysautonomia
•Sensory GBS
two thirds of patients report a preceding event, most frequently an upper
respiratory or gastrointestinal infection Campylobacter jejuni, surgery, or
immunization 1—4 weeks before the onset of neurological symptoms
Treatment
Supportive care in intensive care
Respiratory and bulbar function, the ability to handle secretions, heart rate,
and blood pressure should be closely monitored during the progressive
phase
mechanical ventilation
deterioration in forced vital capacity (FVC),
declining maximal respiratory pressures, and
hypoxemia caused by atelectasis
rapid disease progression (onset to admission in less
than 7 days), bulbar dysfunction, bilateral facial palsies,
and autonomic instability
lower the risks of venous thrombosis and pulmonary embolism
Prevention and prompt treatment of infections
Chest physiotherapy and frequent oral suctioning
regular turning and attention to skin, eyes, mouth, bowel, and bladder
Physical therapy
Psychological support and constant reassurance
Plasma exchange and high-dose intravenous immune globulin(IVIG) infusions have
been shown to be equally effective in moderate to severe weakness
Up to 30% of patients develop respiratory insufficiency requiring assisted
ventilation, and between 2% and 5% die of complications.
20% still have residual motor weakness 1 year later. Approximately 70% of
patients complete their recovery in 12 months and 82% in 24 months.
Chronic Inflammatory Demyelinating polyneuropathy
similar clinical features and CSF change and pathological abnormalities of
multifocal with nerve conduction features reflecting
demyelination. to GBS
The major differences between the two conditions are
in the time course and their response to corticosteroids
Prednisone, plasmapheresis, and IVIg
Multifocal Motor Neuropathy with Conduction Block
more common in men
Progressive, asymmetrical, predominantly distal limb weakness. Profound
weakness in muscles with normal bulk
Treatment with IVIG is currently the preferred treatment
Porphyric Neuropathy
Dominantly inherited disorders
inactivation of one of allelic genes that encodes for an enzyme of the heme
biosynthetic pathway this provokes a compensatory overproduction of porphyrins
and their precursors
Fits + abdominal pain +autonomic manifestations
Only a few patients progress to develop the more ominous motor neuropathy
with generalized, proximal, or asymmetrical muscle weakness developing over
days or weeks