Mayo Clinic 2 - 38 - Myelopathies

Front 1

Define myelopathy

Back 1

Disorder of the spinal cord

Front 2

Most common cause of myelopathy in young patients?

Back 2

MS

Front 3

Most common cause of myelopathy in elderly patients?

Back 3

Cervical spondylotic myelopathy

Front 4

Important features to ask about when working up a myelopathy

Back 4

1. Onset

2. Course

3. In the absence of trauma as an obvious cause, inquire about a history of cancer, infection, immunosuppression, travel history, or residence in certain geographic areas, family history of similar symptoms

Front 5

In addition to neurologic findings, one should also note

Back 5

skeletal abnormalities and skin lesions

Front 6

Acute complete transverse lesions occurring immediately to over a few hours present with what symptoms?

Back 6

1. Spinal shock, that includes paralysis, which is initially flaccid with absent muscle stretch reflexes

2. Loss of sphincter control

3. Sensory level

4. Because of crossing and ascending pain and temperature pathways, the actual spinal cord level of injury is usually a level or 2 higher than the sensory level on examination

Front 7

Central cord syndromes (e.g., caused by a syrinx) can present

Back 7

insidiously with a characteristic capelike loss of pain and temperature with sparing of dorsal column function

Front 8

Later in the course of central cord syndromes

Back 8

Motor neurons and corticospinal tracts are affected, resulting in LMN findings at the level of the lesion, and spasticity below the lesion

Front 9

Clinical picture of ventral cord syndromes

Back 9

One of a complete transverse lesion, except that posterior column function is relatively spared because this is supplied by the posterior spinal artery

Front 10

Clinical picture of posterior column syndromes

Back 10

Posterolateral column syndrome: both a sensory ataxic and spasticity

Isolated posterior column syndrome: sensory ataxia

Front 11

Onset of posterolateral column ysndrome

Back 11

Insidiously, and the classic form is known as subacute combined degeneration, found in metabolic causes of myelopathy or vitamin B12 or copper deficiencies

Front 12

Clinical picture of Brown-Séquard syndrome

Back 12

Loss of pain and temperature contralateral to the lesion and loss of proprioception and weakness ipsilateral to the lesion

Front 13

What may be the initial symptom of a slowly developing compressive lesion at the cervicothoracic junction?

Back 13

Sensory symptoms in the sacral area and in the distal aspect of the legs because the sacral dermatomes are represented more peripherally in the spinothalamic tracts

Front 14

Imaging required if myelopathy has developed over hours to weeks?

Back 14

Yes; should be performed urgently, and the entire spine may have to be imaged, because some causes of compression or inflammation may have multiple sites of disease along the spinal cord

Front 15

Best initial study of spinal cord?

Back 15

MRI; contrast study may be useful if the differential diagnosis includes tumor, infection, inflammation, or certain vascular causes

Front 16

If patients are unable to undergo MRI, what option is the next best?

Back 16

CT myelography

Front 17

Define Lhermitte sign

Back 17

Exacerbation of symptoms with forward flexion of the neck

Front 18

Typical symptoms of demyelinating myelopathy (4)

Back 18

1. Subacute (days to weeks) onset

2. Asymmetric

3. Proprioceptive loss or partial Brown-Séquard syndrome

4. Lhermitte sign is often present

Front 19

Risk of developing MS if at least 2 lesions found on MRI

Back 19

Just under 90% in the next 14 years

Front 20

Presentation of neuromyelitis optica (4)

Back 20

1. Symmetric and severe complete spinal cord syndrome

2. Parapresis

3. Sensory level

4. Bladder and bowel dysfunction

Front 21

MRI findings in neuromyelitis optica (NMO)? (3)

Back 21

1. Longitudinally extensive area of T2 signal change, extending over at least 3 or more vertebral segments

2. Serum NMO immunoglobulin is indicated in this scenario

3. Rule out other causes, such as idiopathic transverse myelitis, sarcoidosis, and myelitis associated with systemic autoimmune diseases

Front 22

By definition, postvaccinal and postinfectious myelitis occur in what timeframe?

Back 22

Within 3 weeks of the vaccination or systemic infection

Front 23

Which vaccines have been most commonly associated with muelitis? (3)

Back 23

1. Hepatitis B

2. Rubella

3. Rabies

Front 24

Postinfectious causes of myelitis? (3)

Back 24

1. Many viruses

2. Mycoplasma

3. Chlamydia

Front 25

Main ways infectious cause myelopathy? (3)

Back 25

1. Abscess (epidural, subdural, rarely intramedullary)

2. Vertebral body destruction with subsequent cord compression

3. Infection of the spinal cord or meninges

Front 26

In patients with known HIV who present with myelopathy, what is the next step in management? (4)

Back 26

1. MRI should be performed to exclude tumor or abscess,

2. Then CSF should be evaluated for bacterial and fungal cultures

3. Studies for viral antigens

4. Likewise, patients with undiagnosed myelopathies should have HIV testing

Front 27

Patients who present with myelopathy acutely to subacutely in the setting of back pain and fever should prompt

Back 27

immediate MRI with and without contrast to exclude spinal epidural abscess

Front 28

Treatment of spinal epidural abscess?

Back 28

Surgical débridement, identification of the organism, and targeted antibiotics

Front 29

How does tuberculosis most commonly cause myelopathy?

Back 29

From tuberculous spondylitis with inflammation from the affected vertebrae putting pressure on the anterior spinal cord, vascular thrombosis, and spinal instability (Gibbus deformity)

Front 30

Treatment of tuberculous myelopathy

Back 30

Surgery and long-term courses of antitubercular drugs

Front 31

Most common way syphilis causes myeliopathy

Back 31

Syphilitic meningomyelitis

Front 32

Time frame for syphilitic meningomyelitis

Back 32

Anywhere from 1 to 30 years after primary infection

Front 33

Presentation of syphilitic meningomyelitis (4)

Back 33

1. Sense of heaviness in the legs with paresthesias

2. Spastic weakness

3. Bladder dysfunction

4. Small-vessel vasculitis may cause spinal cord infarction

Front 34

Tabes dorsalis

Back 34

Form of syphilitic spinal cord disease occurring at least 10 years after the primary infection

Front 35

First phase of tabes dorsalis (5)

Back 35

1. Lightning pain in legs

2. Impotence

3. Bladder dysfunction

4. Areflexic with multimodality sensory loss and Argyll Robertson pupils

5. Lasts about 3 years

Front 36

Second phase of tabes dorsalis

Back 36

1. Lasts between 2 and 10 years

2. Worsening pain and sensory loss, particularly for deep pain and proprioception

Front 37

Last phase of tabes dorsalis (3)

Back 37

1. Lasting 2 to 10 years

2. Significant spastic paraparesis

3. Autonomic dysfunction

Front 38

Two very important points to note about syphilitic myelitis

Back 38

1. Frequent coexistence of syphilis in a patient with HIV

2. Syphilitic meningomyelitis clinically presents very similarly to cervical spondylolytic myelopathy

Front 39

Parasitic myelopathies are common in which areas? (3)

Back 39

1. South America

2. Africa

3. Asia

Front 40

Presentation of parasitic myelopathies (5)

Back 40

1. Acute to subacute onset

2. Leg pain

3. Weakness

4. Sensory loss

5. Bowel and bladder dysfunction

Front 41

When should fungal myelopathies be considered?

Back 41

In immunocompromised patients

Front 42

Which viruses have a predilection for anterior horn cells?

Back 42

Enteroviruses and flaviviruses, they present with flaccid paralysis

Front 43

Spinal cord infarction causes

Back 43

an acute, painful myelopathy

Front 44

Course of spinal cord infarction (3)

Back 44

1. Pain usually occurs first, in a radicular pattern in the lower thoracic or lumbar level

2. Weakness within minutes to hours

3. Bladder and bowel symptoms

Front 45

Prognosis of spinal cord infarction?

Back 45

About half of patients have substantial recovery of function, especially if they have improved motor function in the first 24 hours; often left with considerable central pain syndromes

Front 46

Main risk factors for spinal cord hemorrhage (5)

Back 46

1. Impaired clotting from medications or hematologic disorders

2. Trauma

3. Spinal surgery

4. Lumbar puncture

5. Vascular malformation

Front 47

Most common vascular anomaly of the spine?

Back 47

Spinal dural arteriovenous fistula

Front 48

Back 48

Spinal dural arteriovenous fistula; Sagittal thoracic T2 MRI shows a thoracic-level dural arteriovenous fistula. Note the flow voids dorsal to the cord (arrowhead), representing the fistula. The fistula results in cord edema, evidenced by the high T2 signal (arrow)

Front 49

MRI findings in spinal dural arteriovenous fistula

Back 49

1. Increased T2 signal in the central spinal cord, typically involving the conus and extending longitudinally over 5 to 7 vertebral segments

2. Flow void abnormalities are also found on the surface of the cord

Front 50

Presentation of spinal dural arteriovenous fistula (6)

Back 50

1. No matter the level, the caudal end is affected first by venous congestion

2. Leading to initial sensory-motor symptoms ascending from the feet

3. Gait difficulties

4. Radicular pain

5. Eventually bowel, bladder, and sexual dysfunction

6. May report transient worsening with exercise or prolonged standing

Front 51

Presentation of compressive myelopathies (5)

Back 51

1. Chronically

2. Gait unsteadiness

3. Sense of weakness or stiffness in the legs

4. Difficulty with fine motor skills in the hands

5. Sensory symptoms in the upper and lower extremities

Front 52

Inheritance of hereditary spastic paraplegia can be

Back 52

1. AD (75% to 80% of cases, and one-quarter of these cases are thought to be spontaneous mutations)

2. AR

3. X-linked

Front 53

Clinical features of hereditary spastic paraplegia (3)

Back 53

1. Chronic progressive spastic weakness in the lower extremities

2. May be loss of vibratory function and bladder dysfunction

3. Rate of progression varies considerably between different genetic types and even within families

Front 54

Malformation characterized by a split spinal cord with either a fibrous strand or bony spur running between the 2

Back 54

Diastematomyelia

Front 55

Symptoms of B12 deficiency

Back 55

Initial symptoms are usually paresthesia followed by development of myelopathy

Front 56

Vitamin E deficiency can cause

Back 56

a subacute combined degeneration, but it usually presents as a progressive spionocerebellar syndrome with associated dysarthria and visual symptoms