Disorders of the spine and

spinal cord
Dr OKANGA
Introduction
• Spinal cord contains, in a small cross-sectional area, almost the entire
motor output and sensory input of the trunk and limbs.
• Diseases of the spinal cord are therefore frequently devastating
producing quadriplegia, paraplegia, and sensory deficits.
• Many spinal cord diseases are reversible if recognized and treated at
an early stage.
Cervical spondylosis
• Is the result of osteoarthritis in the cervical spine.
• It is characterised by degeneration of the intervertebral discs and
osteophyte formation.
• Spondylotic changes are common after age 50. Most individuals are
asymptomatic.
• Some affected persons may have neurological dysfunction.
• In order of frequency, the C5/6, C6/7 and C4/5 vertebral levels affect
C6, C7 and C5 roots, respectively,
Cervical radiculopathy
• Causes – Lateral disc prolapse ( acute onset of symptoms), osteophytes
(gradual onset)
• Features – Neck pain that may radiate in the distribution of the affected
nerve root.
• The neck is held rigidly and neck movements may exacerbate pain.
Paraesthesia and sensory loss may be found in the affected segment and
there may be lower motor neuron signs, including weakness, wasting and
reflex impairment.
• Investigations – MRI for patients with radicular symptoms.
• Treatment -analgesics and physiotherapy, discectomy or radicular
decompression
A Anterior. B Posterior
Cervical myelopathy
• Cause - Dorsomedial herniation of a disc and posterior osteophytes
• Mechanism - may result in pressure on the spinal cord or the anterior
spinal artery, which supplies the anterior 2/3 of the cord.
• Features – UMN signs develop in the limbs, with spasticity of the legs
appearing before the arms are involved. Sensory loss in the upper
limbs > lower limbs producing tingling, numbness and proprioception
loss in the hands, with progressive clumsiness.
• Neurological deficit usually progresses gradually and disturbance of
micturition is a very late feature.
• Investigations – MRI C spine.
Cervical myelopathy …
• Management - laminectomy and anterior discectomy, may arrest
progression of disability but neurological improvement is not the rule.
• Prognosis - variable. In many patients the condition stabilises or even
improves without intervention. If progression results in sphincter
dysfunction or pyramidal signs, surgical decompression should be
considered.
Lumbar spondylosis
• Encompasses degenerative disc disease and osteoarthritic change in
the lumbar spine.
• Sciatica (pain in the distribution of lumbar/sacral roots) is usually
caused disc prolapse, ddx - spinal tumour, malignant disease in the
pelvis and TB of the vertebral bodies
Lumbar disc herniation
• Acute lumbar disc herniation is often precipitated by trauma (usually lifting
heavy weights while the spine is flexed),
• Source of pain – Pressure by prolapsed disc on nerve endings in the spinal
ligaments, vertebral joints or nerve roots.
• Pain and spasms of paraspinal alter mechanics of the lumbar spine resulting in
loss of lumbar lordosis.
• Root pressure is suggested by limitation of flexion of the hip on the affected
side if the straight leg is raised (Lasègue’s sign/straight leg raising test SLRT)
• If the L3 or L4 root is involved, SLRT may be negative, but pain in the back may
be induced by hyperextension of the hip (femoral nerve stretch test).
• The roots most frequently affected are S1, L5 and L4;
Clinical features of lumbar herniation
• Onset - may be sudden or gradual.
• Repeated episodes of low back pain may precede sciatica by months
or years.
• Constant aching pain is felt in the lumbar region and may radiate to
the buttock, thigh, calf and foot.
• Pain is exacerbated by coughing or straining but may be relieved by
lying flat.
• Investigation – MRI investigation of choice, Plain x-ray may reveal loss
of lumbar lordosis
Management
• Conservative management ( analgesia and early mobilization) –
effective in 90% of patients.
• Bed rest does not help recovery.
• Advised to avoid physical manoeuvres likely to strain lumbar spine.
• Injections of local anaesthetic or glucocorticoids may be useful
adjunctive treatment if symptoms are due to ligamentous injury or
joint dysfunction
• Surgery – if there is no response to conservative treatment or if
progressive neurological deficits develop
Lumbar canal stenosis
• occurs with a congenitally narrowed lumbar spinal canal, exacerbated
by the degenerative changes that commonly occur with age.
• Pathophysiology - local vascular compromise secondary to the canal
stenosis, renders the nerve roots ischaemic and intolerant of
increased demand that occurs on exercise.
• Age group – Elderly
• Symptoms - exercise-induced weakness and paraesthesia in the legs
(‘spinal claudication’). Symptoms progress with continued exertion,
often to the point that the patient can no longer walk, but are quickly
relieved by a short period of rest.
Lumbar canal stenosis …
• O/E - at rest shows preserved peripheral pulses with absent ankle
reflexes. Weakness or sensory loss may only be apparent if the
patient is examined immediately after exercise.
• Investigations – MRI (choice test) if contraindicated; CT or
myelography.
• Treatment - Laminectomy
Spinal cord compression
• Is a neurological emergency.
• Space-occupying lesion within the spinal canal may damage nerve tissue
either directly by pressure or indirectly by interference with blood supply.
• The early stages of damage are reversible but severely damaged neurons
do not recover – early diagnosis and treatment is key.
• Onset of symptoms is usually slow (over weeks) but can be acute as a
result of trauma or metastases especially if there is associated arterial
occlusion
• Pain and sensory symptoms occur early, while weakness and sphincter
dysfunction are usually late manifestations.
Investigations
• Investigation of choice is MRI
• Plain X-rays may show bony destruction and soft-tissue abnormalities.
Other investigations - chest X-ray, may provide evidence of systemic
disease.
• If myelography is performed, CSF should be taken for analysis; in
cases of complete spinal block, this shows a normal cell count with a
very elevated protein causing yellow discoloration of the fluid (Froin’s
syndrome).
Prostate carcinoma bone metastases
Management
• Treatment and prognosis depend on the nature of the underlying lesion.
• Benign tumours should be surgically excised – prognosis good unless
marked neurological deficit has developed before diagnosis.
• Extradural compression due to malignancy - most common cause of
spinal cord compression in developed countries, prognosis poor Useful
function can be regained if treatment, such as radiotherapy, is initiated
within 24 hours of the onset of severe weakness or sphincter
dysfunction.
• TB Spinal cord compression - may require surgical treatment followed by
appropriate antituberculous chemotherapy.
Intrinsic diseases of the spinal cord
• The most frequent causes of noncompressive acute transverse
myelopathy are spinal cord infarction; systemic inflammatory
disorders, (e.g SLE and sarcoidosis); demyelinating diseases, (e.g
multiple sclerosis and neuromyelitis optica) and postinfectious or
idiopathic transverse myelitis.
• Urinary symptoms usually occur earlier in the course of an intrinsic
cord disorder than with compressive disorder.
• After spinal cord compression is excluded, the evaluation generally
requires a lumbar puncture and a search for underlying systemic
disease.
Evaluation of Acute Transverse
Myelopathy
1.MRI of spinal cord with and without contrast (exclude compressive causes).
2. CSF studies: Cell count, protein, glucose, oligoclonal bands, VDRL; Gram’s
stain, acid-fast bacilli, and India ink stains; PCR for VZV, HSV-2, HSV-1, EBV,
CMV, HHV-6, enteroviruses, HIV; antibody for HTLV-1, Borrelia burgdorferi,
Mycoplasma pneumoniae, and Chlamydia pneumoniae; viral, bacterial,
mycobacterial, and fungal cultures.
3. Blood studies for infection: HIV; RPR; IgG and IgM enterovirus antibody;
IgM mumps, measles, rubella, group B arbovirus, Brucella melitensis,
Chlamydia psittaci, Bartonella henselae, schistosomal antibody; cultures for
B. melitensis. Nasal/pharyngeal/anal cultures for enteroviruses; stool O&P
for Schistosoma ova
4. Systemic immune-mediated disorders: ESR; ANA; ENA; dsDNA;
rheumatoid factor; anti-SSA; anti-SSB, complement levels; antiphospholipid
and anticardiolipin antibodies; p-ANCA; antimicrosomal and
antithyroglobulin antibodies; if Sjogren’s syndrome suspected, Schirmer test,
salivary gland scintigraphy, and salivary/lacrimal gland biopsy
5. Neuromyelitis Optica: Serum anti-aquaporin-4 antibody, anti-MOG
antibody.
6. Demyelinating disease: Brain MRI scan; evoked potentials.
7. Sarcoidosis: Serum angiotensin-converting enzyme; serum Ca; 24-h urine
Ca; chest x-ray; chest CT; total-body gallium scan; lymph node biopsy.
8. Vascular causes: MRI, CT myelogram; spinal angiogram.
Diseases of peripheral nerves
• May affect the motor, sensory or autonomic components, either in isolation or in
combination.
• Causes -systemic diseases, toxins and drugs
• Site of pathology - nerve root (radiculopathy), nerve plexus (plexopathy) or nerve
(neuropathy).
• Presentation - mononeuropathy (single nerve affected), multiple
mononeuropathies (‘mononeuritis multiplex’) or a symmetrical polyneuropathy.
• Pathophysiology - Damage may occur to the nerve cell body (axon) or the myelin
sheath (Schwann cell), leading to axonal or demyelinating neuropathies.
• Only demyelinating neuropathies are usually susceptible to treatment.
• Distinction requires neurophysiology (nerve conduction studies and EMG)
Entrapment neuropathy
• Are caused by compression and/or irritation of peripheral nerves as
they travel through narrow anatomical spaces.
• May affect anyone but diabetes, excess alcohol or toxins, or genetic
syndromes may be predisposing causes.
• Unless axonal loss has occurred, entrapment neuropathies will
recover, provided the primary cause is removed, either by avoiding
the precipitation of activity or by surgical decompression.
Carpal tunnel syndrome
• Most common entrapment neuropathy.
• occurs when the median nerve is squeezed or compressed as it travels
through the wrist.
• characterized by pain in the hand, numbness, and tingling in the distribution
of the median nerve.
• Risk factors for CTS include obesity, monotonous wrist activity, pregnancy,
genetic heredity, and rheumatoid inflammation.
• Pathophysiology - combination of mechanical trauma, increased pressure,
and ischemic damage to the median nerve within the carpal tunnel.
• Treatment - splinting, corticosteroids, surgical decompression in severe cases.
Tinel sign sensitivity 70%, specificity 90% Phalen test sensitivity 66% specificity 88%
Splinting is a first-line treatment for mild to moderate CTS
Local carpal tunnel corticosteroid injection – Symptoms improve
for 10 weeks to up an year
Carpal tunnel decompression surgery provides a lasting, good outcome in 70%
to 90% of cases
Multifocal neuropathy
• Characterised by lesions of multiple nerve roots, peripheral nerves or
cranial nerves.
• Common causes Diabetes and vasculitis.
• Other causes include – Sarcoidosis, Infection (HIV, hepatitis C, Lyme
disease, leprosy, diphtheria), Multifocal motor neuropathy, Multiple
compression neuropathies and lymphoma.
Polyneuropathy
• Multiple mononeuropathy typically affects only a few nerves, often in
different areas of the body. In contrast, polyneuropathy affects
many nerves, usually in about the same areas on both sides of the
body.
• Polyneuropathy is associated with a ‘length-dependent’ pattern,
occurring in the longest peripheral nerves first and affecting the distal
lower limbs before the upper limbs.
• Sensory symptoms and signs develop in an ascending ‘glove and
stocking’ distribution.
Guillain–Barré syndrome
• Is an acute, frequently severe, and fulminant polyradiculoneuropathy that
is autoimmune in nature.
• Males are at slightly higher risk for GBS than females, and adults are more
frequently affected than children.
• Presentation - rapidly evolving areflexic motor paralysis with or without
sensory disturbance. Distal paraesthesia and pain precede muscle
weakness. Weakness is severe proximally than distally.
• Facial and bulbar weakness commonly develops, and respiratory weakness
requiring ventilatory support occurs in 20% of cases.
• Weakness progresses over a maximum of 4 weeks (usually less). Rapid
deterioration to respiratory failure can develop within hours.
Antecedent Events - GBS
• Approximately 70% of cases of GBS occur 1–3 weeks after an acute
infectious process, usually respiratory or gastrointestinal.
• Implicated pathogens include - Campylobacter jejuni, CMV, EBV, HIV,
hepatitis E, Zika and Mycoplasma pneumoniae
• GBS may occur after immunization,
• GBS also occurs more frequently than can be attributed to chance
alone in patients with lymphoma (including Hodgkin’s disease), in HIV-
seropositive individuals, and in patients with systemic lupus
erythematosus (SLE).
-GBS
• Immunopathogenesis - All GBS results from immune responses to
nonself antigens (infectious agents, vaccines) that misdirect to host
nerve tissue (molecular mimicry)
• Investigations - CSF protein↑(may be normal in the first 10 days). CSF
white cell count - Normal (Counts > 10 × cells/L suggests an alternative
diagnosis)
• Management - plasma exchange or intravenous immunoglobulin
therapy shortens the duration of ventilation and improves prognosis.
• Prognosis - 80% of patients recover completely within 3–6 months, 4%
die and the remainder suffer residual neurological disability, which can
be severe.
Chronic polyneuropathy
Charcot-Marie-Tooth disease
• (CMT) is a group of inherited conditions that damage the peripheral
nerves.
• Cause - inherited mutation in one of the many genes responsible for
the development of the peripheral nerves
• Symptoms - muscle weakness feet, ankles, legs and hands, an
abnormal gait, highly arched or very flat feet, numbness in the feet,
arms and hand
inverted champagne bottle legs.
High arches (pes cavus) Flat feet (pes planus)
• https://www.standardmedia.co.ke/sunday-magazine/article/
2001331007/my-battle-with-rare-guillain-barre-syndrome