Nerve diseases Flashcards
What is peripheral neuropathy + name types of peripheral neuropathy
Damage of peripheral nerves
Can affect one nerve (mononeuropathy), two or more nerves in different areas (multiple mononeuropathy) or many nerves (polyneuropathy)
Causes of peripheral neuropathy (8)
Heriditary Diabetes Alcoholism Infectious diseases - lyme, HIV Certain medications - esp chemo Tumour compression Paraneoplastic - body's immune response as a result of the cancer Autoimmune diseases - Guillain barre syndrome, chronic inflammatory demyelinating polyneuropathy
Pathophysiology of Guillain barre syndrome (acute inflammatory demyelinating polyneuropathy)
-proposed mechanism
Acute inflammatory demyelinating polyneuropathy (i.e. a peripheral neuropathy affecting many nerves)
Thought to maybe be an AUTOIMMUNE ATTACK OF MYELIN sheath of peripheral sensory and motor nerves –> decreased propagation of electrical impulses –> motor weakness/flaccid paralysis
When immune reaction stops, repair and remyelination promptly begin, which correlate with a quick recovery from the flaccid paralysis
Risk factor of Guillain barre syndrome
Preceding viral/bacterial infection, esp gastroenteritis or URTI
Clinical features of Guillain barre syndrome
- which symptoms are seen first
- sensory (1)
- motor (8)
(hint: bilateral, FLACCID, ascending weakness of limbs peaking in <4 weeks)
Paraesthesia - PRECEDES WEAKNESS
Back and leg pain - PRECEDES WEAKNESS
Muscle weakness
- usually hands/feet first BEFORE spreading to arms/legs
- facial weakness
- oropharyngeal weakness –> dysphagia
- extraocular muscle weakness –> diplopia
- ptosis
- dysarthria
- dyspnoea/resp distress
Absent reflexes/hyporeflexia
FLACCID PARALYSIS (late symptom)
Investigations of Guillain barre syndrome + their findings (2)
Nerve conduction studies - shows slowing of conduction velocities
LP - shows increased CSF protein but NORMAL CELL COUNT
Where do symptoms of peripheral neuropathies usually start
Distally in feet and hands and spread up legs/arms
UMN lesion signs (4) + 2 not seen
Weakness NO WASTING Increased tone (spasticity) Brisk/exaggerated reflexes NO FASICULATIONS Babinski +ve (extensor plantar)
LMN lesion signs (6)
Weakness Wasting Decreased tone (flaccidity) Decreased reflexes Fasciculations Babinski -ve (flexor plantar)
Symptoms (3) /signs (10) of motor neurone disease (amyotrophic lateral sclerosis)
BOTH UMN (4) + LMN SIGNS (6)
Symptoms:
Dyspnoea
Dysphagia
Dysarthria
Motor neurone disease is a disorder of what horn
Anterior (Ventral) horn
Muscle weakness usually begins in the (1) in motor neurone disease but sometimes may begin in other places - name these (2)
Limb weakness
May also begin in bulbar muscles (Swallowing + speech) or resp muscles
What initial presentation of motor neurone disease is more aggressive - limb weakness or bulbar weakness
Bulbar weakness
Investigations/diagnosis (2) of motor neurone disease
Clinical diagnosis is 1st line - a thorough history and examination
Electromyography (EMG) - shows diffuse, ongoing, chronic denervation
Treatment of motor neurone disease
- supportive
- medical
Supportive/palliative care
- mechanical ventilation
- physio
- speech and language therapy
- feeding tube
Riluzole - prolongs survival as it’s a glutamate antagonist (degeneration in ALS is caused by excitatory glutamate neurotransmitter so if you block that then can slow degeneration)