Neuropathies and entrapments

Cranial Nerves

Olfactory I smell from mucosa of roof of nasal cavity
Optic II sight - retina
Oculomotor III motor to sup, inf, med recti, inf oblique, levator palpebrae superioris
parasymp to sphincter pupillae and ciliary M's - constriction and accommodation
Trochlear IV sup oblique - inf gaze
Trigeminal V
Trigeminal Neuralgia
Ophthalmic Va sensation - eyelids, scalp, nose and nasal cavity, paranasal sinuses
Maxillary Vb sensation - maxillary face, upper lip, upper teeth, maxillary sinuses and palate
Mandibular Vc sensation - mandibular face, lower teeth, ant 2/3 tongue
motor - mastication
Abducens VI motor - lat rectus - lat gaze
Facial VII
Bells palsy
Ramsay Hunt
motor - facial M's and middle ear
parasymp - salivary and lacrimal glands, glands of nose and palate
sensory - taste ant 2/3 tongue and palate
Vestibulocochlear VIII Vestibular - semicircular canal sensation, saccules - position
Cochlear - hearing
Glossopharyngeal IX Sensory - ext ear
Motor - stylopharyngeus (swallowing)
parasymp - parotid
Taste - post 1/3 tongue
sensory - parotid, carotid body/sinus
Vagus X Motor - pharynx, larynx, upper 2/3 oesophagus
Parasymp - cardioresp, gut
Visceral sensory - thorax, abdomen
taste - epiglottis and palate
Sensation - ext auricle, ext auditory meatus, dura of post cranial fossa
Accessory XI Motor - SCM and trapezius
Hypoglossal XII Motor - tongue

Bells Palsy (CN VII)

  • rapid (<72/24) onset of facial paralysis - may be preceded by a slight fever, pain behind the ear on the affected side, a stiff neck, and weakness and/or stiffness on one side of the face.
  • unilateral paralysis is complete vs stroke which does NOT include forehead paralysis due to crossing of innervation
  • may have loss of taste on one side of mouth, drooling, and an increased sensitivity to sound (hyperacusis)
  • usually results from ischemia and/or compression of CN VII
  • exact cause not known - viral and auto-immune frequently implicated. There appears to be an inherited tendency
  • usually resolves - 2/52
  • Prednisolone if presents ≤72/24. No consensus on dosage but 2 possible regimens:
    • 50 mg daily for 10/7 or
    • 60 mg daily for 5/7 followed by a daily reduction in dose of 10 mg (for a total treatment time of 10/7) if a reducing dose is preferred.
  • Differential:
    • acoustic neuroma - due to associated compression of the neuroma on VII
    • Myasthenia Gravis
    • Ramsay-Hunt syndrome - due to Varicella zoster. Look for associated rash

House-Brackmann score for the Facial nerve

I. Normal Normal facial function in all areas
II. Mild dysfunction Gross
Slight weakness noticeable on close inspection
May have slight synkinesis
At rest, normal symmetry and tone
Motion
Forehead - Moderate to good function
Eye - Complete closure with minimal effort
Mouth - Slight asymmetry
III. Moderate dysfunction Gross
Obvious but not disfiguring difference between sides
Noticeable (but not severe) synkinesis, contracture, or hemifacial spasm
At rest, normal symmetry and tone
Motion
Forehead - Slight to moderate movement
Eye - Complete closure with effort
Mouth - Slightly weak with maximum effort
IV. Moderately severe dysfunction Gross
Obvious weakness and/or disfiguring asymmetry
At rest, normal symmetry and tone
Motion
Forehead - None
Eye - Incomplete closure
Mouth - Asymmetrical with maximum effort
V. Severe dysfunction Gross
Only barely perceptible motion
At rest, asymmetry
Motion
Forehead - None
Eye - Incomplete closure
Mouth - Slight movement
VI. Total paralysis No movement

Ramsay Hunt syndrome (CN VII)

rare neurological disorder caused by varicella zoster characterized by paralysis of CN-VII with variable presentation of other findings:

  • erythematous & vesicular rash affecting the pinna and sometimes external auditory canal and occasionally the soft palate and pharynx.
  • may be associated with tinnitus and hearing loss.
  • Treatment with antivirals ≤72/24 + other symptomatic Rx

Thoracic outlet Syndrome

  • Compression of the brachial plexus which can occur between the anterior and middle scalene muscles, underneath a cervical rib, beneath the pectoralis minor tendon, or by fibrous bands anywhere along the course of the plexus.
  • scalenus anticus syndrome: abnormal insertion of scalenus anterior onto 1st rib (commonest cause)
  • congenital cervical rib
  • bony abnormality of 1st rib or clavicle (congenital anomaly, malunited fracture, callus, Paget disease, tumour)
  • elongated C7 transverse process
  • muscle hypertrophy
  • fibrous bands
  • supraclavicular tumour or lymphadenopathy
  • Differentials - other causes of brachial plexopathy including trauma, mass lesions, vascular lesions, brachial plexitis, and cervical spinal cord or foraminal stenosis

subtypes:

  • arterial thoracic outlet syndrome (ATOS)
  • neurogenic thoracic outlet syndrome (NTOS)
  • venous thoracic outlet syndrome (VTOS)
  • neurogenic pectoralis minor Syndrome (NPMS)
  • venous pectoralis minor syndrome (VPMS)
  • arterial pectoralis minor syndrome (APMS)

Neurogenic TOS (95% of all TOS) presents with pain, weakness, numbness and tingling in the hand and arm.

  • neck pain and headache in the back of the head are common.
  • usually caused by neck trauma - whiplash injuries or repetitive stress injury at work commonly initiate symptoms. The original injury said to cause over-stretching of the neck muscles which heal by forming irritative scar tissue

Venous TOS, also known as Paget-Schroetter disease, presents with arm swelling, blue or dark discolouration, and a feeling of fullness or aching in the arm

  • usually an anatomical variation leading to compression or thrombosis (Paget-Schroetter disease)

Arterial TOS presents with coldness, numbness, tingling, pain, and white discolouration in the fingers or whole hand.

  • Cramping of the forearm and hand with activity (claudication) is common.
  • Pain usually involves the hand and arm, but not the neck or shoulder.
  • more commonly related to thrombus

Useful tests:

  • Wright test - hyperabduction leading to increased pain or loss of pulse in vascular TOS
  • Adson test - ipsilateral head rotation and inspiration leading to increased pain or loss of pulse in vascular TOS.

Brachial plexus

Brachial Neuritis

(Parsonage-Turner Syndrome)

  • otherwise known as idiopathic or hereditary neuralgic amyotrophy
  • uncommon, characterized by severe shoulder pain followed by patchy muscle paralysis and sensory loss involving the shoulder girdle and upper extremity.
  • any nerve or branch within the brachial plexus can be involved
  • auto-immune and biomechanical causes implicated
  • ultimately inflammatory processes predominate
  • lower trunk worse prognosis c/w upper trunk
  • recovery over many months - 2/3 have motor function ≤1/12
Presentation
  • Often in stages
    • sudden severe pain - may last weeks
    • painless flaccid paralysis - more often upper branches
    • slow recovery over months - 6-18/12 recovery period for motor function
Differential Diagnosis
  • Cervical spine radiculopathy - pain and weakness follows a specific nerve root distribution
  • Rotator cuff pathology - shoulder pain persists despite development of shoulder weakness
  • Entrapment neuropathy - shoulder pain with progressive weakness in a specific peripheral nerve distribution
  • Idiopathic hypertrophic brachial neuritis (IHBN) - rare and painless. Characterized by weakness in upper limb muscles and hypertrophy of the brachial plexus

Lumbar plexus nerve entrapments

  • Groin pain of neural origin can be caused by pelvic surgeries, blunt trauma, sports injury, hypertrophy of the abdominal muscles from excessive training, or from unknown causes
  • genitofemoral and lateral femoral cutaneous nerve entrapments can result from tight clothing
  • Obturator nerve entrapment can be caused by pelvic trauma and tumours or normal pregnancy
  • Among athletes, most cases of entrapment of the ilioinguinal nerve are linked to hypertrophy of the abdominal muscles as a result of excessive training
  • Iliohypogastric nerve injury combined with tears in the external oblique aponeurosis has been described in soccer and rugby players
  • In athletes, obturator nerve entrapment appears to be the result of a fascial band at the distal end of the obturator canal, esp in skaters, Australian Rules football players, and soccer players as a result of adductor muscle development

Beriberi

a condition due to deficiency of Thiamine (vit B1)

  • Dry Beriberi - neurological symptoms
  • Wet Beriberi - Cardiac symptoms with or without neurology
  • infantile Beriberi - neurocardiac symptoms in infant <1/12
  • sensorimotor neuropathy of dry Beriberi can mimic the signs and symptoms of acute or chronic GBS.
  • mild-to-moderate reduction in all sensory modalities in a stocking distribution with reduced reflexes.
  • may have mild, predominantly distal weakness.
  • Wet beriberi can present with fulminant CVS collapse with overload and severe lactic acidosis which rapidly responds to Thiamine replacement
  • Thiamine deficiency:
    • reduced thiamine intake - alcohol abuse, anorexia nervosa, dieting, or malabsorption following bariatric surgery
    • Malabsorption - diarrhea, celiac disease, tropical sprue, or dysentery.
    • Other - burns, pregnancy, dialysis, and malignancy
  • Thiamine repletion leads to rapid clinical improvement in dry beriberi c/w Guillain Barré Syndrome

Cauda Equina Syndrome

Red flags:

  • Severe low back pain (LBP)
  • Sciatica: often bilateral but sometimes absent, especially at L5/S1 with an inferior sequestration
  • Saddle and/or genital sensory disturbance
  • Bladder, bowel and sexual dysfunction
  • Mostly commonly large lumbar disc herniation
  • Less common - epidural haematoma, infections, malignancy, trauma

3 main variations:

  1. Rapid onset without a previous history of back problems.
  2. Acute bladder dysfunction with a history of low back pain and sciatica.
  3. Chronic backache and sciatica with gradually progressing CES often with canal stenosis.

Important to distinguish between Complete and Incomplete as CES-Incomplete has a good prognosis with emergency surgical intervention. CES-Complete does not respond as well and surgery may even be delayed:

  • CES-Incomplete
    • urinary difficulties of neurogenic origin including altered urinary sensation
    • loss of desire to void, poor urinary stream and the need to strain in order to micturate
    • Saddle and genital sensory deficit is often unilateral or partial
    • trigone sensationclose an inflated urinary catheter is gently pulled and should elicit the desire to micturate should be present.
  • CES-Complete
    • painless urinary retention and overflow incontinence (CES-Retention)
    • There is usually extensive or complete saddle and genital sensory deficit
    • deficient trigone sensation.

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