Neuropathies and entrapments
Cranial Nerves
Olfactory I | smell from mucosa of roof of nasal cavity |
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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 |
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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:
- Rapid onset without a previous history of back problems.
- Acute bladder dysfunction with a history of low back pain and sciatica.
- 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 sensation 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.
References include:
Lumbar Plexus N entrapment syndromes - groin pain in athletes
https://www.orthobullets.com/shoulder-and-elbow/3065/brachial-neuritis-parsonage-turner-syndrome
https://basicmedicalkey.com/summary-of-cranial-nerves/
https://cks.nice.org.uk/topics/bells-palsy/
Cauda equina review 2011
https://rarediseases.org/rare-diseases/thoracic-outlet-syndrome/
J NeuroSurg. Thoracic Outlet synd 2015