Peripheral nerve 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 stocking- glove pattern.

Classification Acute Subacute Chronic Small nerve fibers Large nerve fibers Axonal Demylination Mixed Classification

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-Marie- Tooth 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 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, 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. Clinical features

GBS Types Acute inflammatory demyelinaring polyradiculoneuropathy Acute motor axonal neuropathy Acute motor sensory axonal neuropathy Millar-Fisher syndrome ( ophthalmoplegia, ataxia and areflexia ) 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 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 Dominantly inherited disorders 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