History and exam

Key diagnostic factors

common

presence of risk factors

Common risk factors include recent infectious illness and vaccination.[1][34]

age 10-19 or 30-39 years

Bimodal age distribution for idiopathic TM.[1][23]

motor weakness

Progressive weakness involving the lower extremities or all extremities. More than 80% of patients with definite or probable idiopathic acute TM have a motor deficit at presentation.[24]

Weakness follows a pyramidal pattern (arm abduction; elbow, wrist, and digit extension; hip and knee flexion; and ankle dorsiflexion), is accompanied by upper motor neuron signs such as hyper-reflexia and spasticity, and can reach its nadir at any time from 24 hours to 4 weeks.[1][34]

Very acute cases may be associated with flaccidity and areflexia early in the course ('spinal shock').

paraesthesias or sensory loss

Concomitant with motor weakness, most patients develop ascending tingling and/or numbness, beginning in the distal limbs and settling at or a few levels below the spinal lesion level.[1][2]

About 75% of patients with definite or probable idiopathic acute TM have a demonstrable sensory deficit at presentation.[24]

bladder symptoms: urinary frequency, urgency, incontinence, or retention

Urinary disturbance affects >60% of patients with definite or probable idiopathic acute TM.[24] This may present as urinary frequency, urgency, incontinence, or retention.

bowel symptoms: incontinence or constipation

Symptoms are variable but include bowel incontinence or constipation.

L'hermitte sign

A hallmark of demyelination, L'hermitte sign consists of paraesthesias (tingling, an 'electrical sensation') in the limbs, elicited by neck flexion.[52]

McArdle's sign

A hallmark of demyelination. Consists of an increase in the degree of pyramidal weakness with neck flexion.[53]

paroxysmal tonic spasms

Recurrent, stereotypical, painful, involuntary motor spasms of ≥1 limbs, each lasting 15 to 45 seconds.[54]

These may be mistaken for focal motor seizures and can recur tens of times daily.

upper motor neuron signs: hyper-reflexia, positive Babinski's sign, limb spasticity

Motor weakness is typically associated with hyper-reflexia, Babinski's signs, and limb spasticity.[1]

sensory loss/sensory level

Reduced pain and temperature sensation at or a few levels below the level of the spinal cord lesion, either unilateral or bilateral.[1][2]

Involvement of the posterior columns results in impairment of vibratory and proprioceptive sensation in the distal limbs below the lesion level.

uncommon

dyspnoea/respiratory distress

Involvement of the upper cervical cord may cause neurogenic respiratory failure. This presents as dyspnoea and subjective respiratory distress and is more common in longitudinally extensive TM than in acute partial TM.[4][57]

Other diagnostic factors

common

back pain

Mid-line back pain at the approximate level of the spinal cord lesion affects at least one-third of adults and 75% of children with TM.[34][51]

When present, the pain is usually constant and moderately severe.

trunk/limb pain

Shooting, lancinating, burning, or similar discomfort occurs in a segmental distribution because of involvement of the root entry zone at the level of the lesion.

areflexia/hyporeflexia

Very acute and severe TM may be associated with 'spinal shock', which is recognised by flaccid paralysis and reduced or absent tendon stretch reflexes.[11] This generally evolves into a typical spastic paraparesis/quadriparesis pattern with hyper-reflexia and Babinski signs.

uncommon

hiccups

Longitudinally extensive TM may extend into the medulla and cause persistent hiccups.[55][56]

nausea/vomiting

Longitudinally extensive TM is associated with persistent nausea and vomiting, probably owing to involvement of the area postrema.[4][56]

Risk factors

strong

preceding infectious illness

More than half of patients, regardless of age, have had a systemic viral illness within 1 to 4 weeks prior to onset of neurological symptoms, especially in children.[11][34]

recent vaccination

Post-immunisation myelitis has been reported with almost all vaccinations.[33][34]

female sex

Demyelinating diseases and almost all other autoimmune diseases associated with TM have a female predilection.[18][24]

weak

history of recent physical trauma

Cases of inflammatory myelitis following physical trauma have been reported, but the relationship is unclear.[34]

spinal injection

Case reports describe TM occurring in temporal association with spinal epidural anaesthesia or lumbar spine corticosteroid injection.[35][36]

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