periphera l neuropathy Flashcards
ANTERIOR HORN CELL DISEASES
PRIMARY disease of motor neurons ; degenerative as ; MND AND SMA
infection; poliomyltitis
spinal cord pathology [ ahc + other pat of cord]
vascular ; anterio spinal artery ischemia
mechanical ; syringomyleia
what is motor neuron disease
it is mixed upper and lower motor neuron affection
MND TYPES
most common type ; als which affect umn and lmn
primary musclar atrophy ; pure lower motor
primary lateral sclerosis ; pure umn
what is the most common type of MND
ALS ; MIXED UMN AND LMN
TREATMENT OF MND
SUPPORTIVE AND RILUZOLE
HOW TO DIAGNOSE MND
CLINICAL DIAGNOSIS , NCS . EMG
DD OF DISEASE THAT MAKE UMN AND LMN SIGN
FRIEDRCHS ATAXIA
MND
CERVICAL SPONDYOLYSIS
CONUS MEDULLARIS
SUBACUTE COMBINED DEGENRATION OF THE CORD [ VIT 12 DEFECIENCY]
TABOPARESIS [ SYPHYLIS]
RADICULOPATHY causes
mechanical :
degenerative as spondyolysis (pars intercularis fractuer which is connect 2 joints together )
mass lesion :bone metastsis
trauma
medical :
inflammatory as Gbs
neoplastic i filtrate
post radiotherapy
degenerative spine diseases types
compress on the roots :Lmnl
compress on the centeal canal :Umnl
cimpress on both : mixed Umnl and Lmnl
Signs & Symptoms of
Radiculopathy
Dermatomal/myotomal distribution
* PAIN
– Locally in neck / back
– Referred to dermatome
* Numbness, tingling – dermatomal.
* Weakness (myotome distribution)
– But sometimes purely sensory.
* Reduced reflexes depending on affected root
CAUDA EQUINA SYNDROME
LUMBOSACRAL AFFECTION
CLINICAL PRESENTATION OF CAUDA EQUINA SYNDROME
Sacral roots saddle anaesthesia, urinary retention (flaccid bladder, volume bladder),
impotence,
– Often absent reflexes in legs (lumbar roots
SADDLE ANATHESIS
“Saddle anesthesia”—numbness and loss of sensation in the buttocks, perineum, and inner surfaces of the thighs
(MYELOPATHY gives a sensory LEVEL ie not dermatomal, spastic bladder (small
volumes, empties without warning) , UMN signs below the level of the lesion)
INVESTIGATION of radiculopathy
imaging;
CT – good for bones. Can’t see nerve tissue.
– MRI – bones + signal change in neural elements
Nerve conduction & EMG
– Confirm denervation in a root distribution
– Normal sensory action potentials despite
numbness
INVESTIGATION of radiculopathy
imaging;
CT – good for bones. Can’t see nerve tissue.
– MRI – bones + signal change in neural elements
Nerve conduction & EMG
– Confirm denervation in a root distribution
– Normal sensory action potentials despite
numbness
management of radiculopathy
Pain control
* Spondylosis
– Orthopaedic or neurosurgical opinion
– Surgery to prevent worsening (may not make
weakness better once muscle has wasted).
* Medical causes
– Treat underlying cause
neuropathy causes
Metabolic (DM, B12 defic., etc)
– Toxic (alcohol, drugs – e.g.
chemotherapy)
– Infection eg. HIV, Lyme, Syphillis
– Compression
– Autoimmune
– Degenerative (idiopathic axonal
neuropathy)
– Hereditary (CMT
Hoarse voice, wasted hand, weight loss, smoker
what is the disease
pancoast tumor
Funny rash, tickbite, arthralgia, heartblock, cranial nerve palsy and radicular pains
what is the disease ?
lyme disease
what is the disease ; crohns affecting terminal ileum and polyneuropathy
b 12 defeciency
what type of neuropathy ?
- What is the DISTRIBUTION? (individual nerve(s) / all the longest nerves)
- Acute or chronic? relapsing and remitting? Slowly or rapidly progressing?
- Axonal / demyelinating (ask for nerve conduction study)
- Motor fibres / sensory fibres / both
- Large fibres / small fibres / both (burning and pain = small fibre)
– Large fibres – check proprioception, light touch, vibration
– Small fibres – check pain (pin), temperature (cold tuning fork) - Autonomic involvement?
– Postural hypotension, sweating too much/little, impotence, gastrointestinal
symptoms, pupillary abnormalities - Cranial nerve involvement?
- Family history? – any chance that parents are related?
long nerve affection ? [many nerve affection]
- Polyneuropathy
- Longest nerve endings die back
- Feet then legs then hands
- Glove & stocking
- Symmetrical
- Long list of causes
– B12, Folate
– Diabetes & thyroid dysfunction
– Drugs & toxins
– Alcohol
– Infections eg HIV/AIDS
– Critical illness neuropathy
– Connective tissue diseases egRA, SLE,
ChurgStrauss
– Carcinomatous neuropathy
individual nerve affection?
- Single nerve (mononeuropathy)
– Compression: - Median at the carpal tunnel
- Ulnar at the elbow
- Common Peroneal at the fibular head
- Radial in spiral groove of humerus
– Idiopathic eg Bell’s palsy
– Inflammatory
– Infiltration by tumour - Several nerves (monon. mul)
- Patchy, asymmetrical
– VASCULITIS (painful)
– INFLAMMATORY
– DIABETES (causes a vasculitis) - Hereditary neuropathy with liability to
Pressure Palsies (rare)
causes of mylein sheath degeneration
- Hereditary(rare) * – CMT type 1
- Inflammatory/autoimmune – GuillainBarre Syndrome
– Chronic inflammatory demyelinating polyneuropathy (CIDP)
– Multifocal motor neuropathy
– Paraproteinaemic neuropathy
motor affection only / what the disease ?
Multifocal motor neuropathy (MMN)
– ALWAYS PURELY MOTOR
– Treatable but can be mistaken for
MND.
* GuillainBarre Syndrome
* CIDP
* CharcotMarieTooth
– Can be mainly motor
* Rarities:
– Lead, Arsenic, Thallium
– Porphyria
– Diptheria
mainly sensory nerve affection disease ?
Causes of pure sensory neuropathy
– Paraneoplastic
– Drugs
– Carcinomatous sensory neuronopathy
– Lymphomatous sensory neuronopathy
– Sjögren’s syndrome
– Paraproteinemias
– Nonsystemic vasculitic neuropathy
– Primary biliary cirrhosis
– Crohn’s disease
– Chronic gluten enteropathy
– Hereditary sensory neuropathy
lenght dependent sensory neuropathy
most common pattern
typically axon
most commnly in diabetic , alcholo both are painful or idiopathic
treat underlyine cause
medication of lenght dependnt sensory neuropathy
for pain as gabentin , amitryptilline and pregablain
small fiber neuropathy ?
- Painful, burning
- Nerve conduction normal unless coexisting large
fibre neuropathy - Measure temperature perception (thermal threshold)
- Diabetic neuropathy
Alcoholic neuropathy
Amyloidosis
HIV/AIDS - Leprosy
- Hereditary
diseases that involve autonomic
- Diabetic neuropathy Alcoholic
neuropathy HIV/AIDS - GuillainBarré syndrome Amyloidosis
Paraneoplastic neuropathy Lymphoma - Thiamine deficiency
- Porphyria
- Thallium, arsenic, mercury toxicit
cidp
Chronic version of GBS (>8 weeks form onset to peak; can be insidious)
* Often relapsing-remitting course
* Presumed autoimmune but no identified cause
* Responds to treatment (steroids, other oral immunosuppressants, IVIg,
plasma excahnge)
* Patients my need prolonged / cycles of treatm
charcot marie tooth 1
Pes cavus, inverted champagne bottle legs
– Autosomal dominant demyelinating neuropathy due to
duplication of PMP22 gene (myelin constituent)
– Insidious onset
– Progressive distal weakness often without subjective
sensory loss (but absent sensory responses on NCS)
– Very slow nerve conduction
– DNA testing allows genetic counselling and selective IVF
(only implant embryos without mutation)
