AFECTIUNILE MEDULARE.

Prof. Mihail GAVRILIUC

AFECTIUNILE MEDULARE

Frecvent sunt devastatoare.

Pot produce para- / tetraplegie, tulburri de

sensibilitate, dereglri sfincteriene.

Multe din ele sunt reversibile i tratabile n

cazul iniierii unui tratament precoce.
AFECTIUNILE MEDULARE TRATABILE

COMPRESI VASCULAR INFLAMATO INFECTIO DE METABOL

VE E RII ASE DEZVOLTARE ICE
Neoplasmele MALFORMATIA Scleroza Virale: VZV, Siringomielia Deficient
(epidurale, ARTERIO- Multipl HSV-1 and -2, de vitamin
intradurale, VENOAS CMV, HIV, Meningomielocel B12
intramedulare) (MAV) Neuromielita HTLV-I, e (degenerare
optic others subacut
Abcesul Sindromul Tethered cord combinat)
epidural antifosfolipidic Mielita Bacteriale si syndrome
si alte stri de transvers mycobacteria Carenta de
Hemoragia hipercoagulabi le: Borrelia, cupru
epidural litate Sarcoidoza Listeria,
syphilis,
Spondiloza Vasculita altele
cervical
Mycoplasma
Hernia de disc pneumoniae
intrevertebral
Parazitiare:
Compresia schistosomia
post- za,
traumatic toxoplasmoz
a
Note: VZV, varicella-zoster virus; HSV, herpes simplex virus; CMV, cytomegalovirus; HTLV, human T cell lymphotropic virus.
 SINDROMUL DE NEURON MOTOR
PERIFERIC
este pareza/plegia cauzat de
lezarea neuronului motor
periferic
la nivelul uneia din formaiunile
anatomice care
formeaz calea spino-
muscular:

- corn medular anterior,

- rdcin medular anterioar,
- nerv spinal,
- plex,
- nerv periferic motoriu,
- sinaps
 SINDROMUL DE NEURON MOTOR
PERIFERIC
Poate cuprinde urmtoarele manifestri clinice:

- hipotrofie / atrofie muscular;

- fasciculaii musculare;

- hipotonie / atonie muscular;

- hiporeflexie / areflexie.
 SINDROMUL DE NEURON MOTOR
PERIFERIC
Manifestrile electrofiziologice:

Activitatea muscular spontan patologic

de denervare, nregistrat n cadrul
examenului electromiografic cu ac-electrod
i care const din:

- poteniale de fibrilaii,
- fasciculaii
- i unde pozitive ascuite.
 ENTITI NOZOLOGICE NSOITE DE
SINDROMUL DE NEURON MOTOR
PERIFERIC
LEZIUNI MEDULARE:
mielit de divers etiologie;
poliomielit;
scleroza lateral amiotrofic;
scleroza multipl;
ictus medular;
mielopatie vascular cronic;
siringomielie; contuzie, comoie, compresie
medular traumatic;
tumori extra- i intramedulare etc.;
Transverse section through the
spinal cord

the principal the principal

ascending descending
pathways pathways
(left) (right)

The lateral and ventral spinothalamic tracts (blue) ascend contralateral to the side of the body that is innervated. C, cervical;
T, thoracic; L, lumbar; S, sacral; P, proximal; D, distal; F, flexors, E extensors.
SPINAL CORD LEVELS RELATIVE TO THE
VERTEBRAL BODIES

SPINAL CORD LEVEL CORRESPONDING VERTEBRAL

BODY
Upper cervical Same as cord level

Lower cervical 1 level higher

Upper thoracic 2 levels higher

Lower thoracic 2 to 3 levels higher

Lumbar T10-T12

Sacral T12-L1
 NOIUNI ANATOMICE FIZIOLOGICE DESPRE
FUNCIA DE CONTINEN A URINEI

Vezica urinar este constituit

din muchiul detrusor
(muchi neted), sfincter
intern al urinei (muchi
neted) i sfincter extern al
uretrei (muchi striat).
Funcia muchilor netezi
este controlat de sistemul
nervos vegetativ (autonom),
iar funcia muchiului striat
este condus de sistemul
nervos animalic (somatic).
TULBURRI DE CONTINEN A
URINEI

TULBURRI SFINCTERIENE DE TIP CENTRAL

TULBURRI SFINCTERIENE DE TIP PERIFERIC

SINDROMUL BROWN-SQUARD
MIELITA

Este o boal infecioas acut de focar a

mduvei spinrii deseori cu lezare
transvers att a substanei albe ct i a
celei cenuii, uneori cu implicare a
rdcinilor i nveliurilor medulare. n
funcie de criteriul patogenic mielitele
sunt primare i secundare.
POLIOMIELITA ACUT ANTERIOAR (paralizia
medular infantil, boala Heine - Medin)

ETIOLOGIE.
Virusul poliomielitic face parte din familia
Picornaviridae, genul Enterovirus. Este
un virus foarte mic (28 nm), cu 3 tipuri
antigenic distincte: tipul 1 (diverse
tulpini: Brunhilde, Mahoney etc.), tipul 2
(Lansing) i tipul 3 (Leon, Saukett).
POLIOMIELITA. Patogenie.
 AnatomiaVascularSpinal
n primele sptmni ale
dezvoltrii umane embrionare are
loc realizarea paternului
segmentar de vascularizare
spinal: de la poriunea dorsal a a
aortei pornesc artere pare ctre
fiecare din cei 31 de de somii.
Conform acestui model metameric
se vascularizeaz corpul vertebral,
nveliul dur, nervii spinali, mduva
Nett spinrii (poriunea dorsal,
er ventral i central).

n perioada postnatal mduva

spinrii se alimenteaz cu snge
Thro de ctre artera spinal anterioar
n
i dou artere spinale posterioare
care sunt un canal anastomotic al
ramurilor ascendente i
ArtereleMduveiSpinrii

1 artera radiculomedular, 2 artera radicular anterioar,

3 artera radicular posterioar, 4 artera spinal
anterioar(ASA), 5 artera spinal posterolateral(ASPL), 6
artera spinal posterioar, 7 reeaua longitudinal pial
discontinu, care d natere ramurilor perforante ale
vasei corona, 8 arterele sulcale (centrale)
Thron AK. Vascular Anatomy of the Spinal Cord. 1988.
Legea ultimului cmp n patogenia leziunilor
vasculare medulare.
Zonele critice de irigare sanguin medular deficitar
Patologia vascular medular
Poate fi:
1. Tip ischemic: acut
cronic
2. Tip hemoragic hematomielia

Focarul ischemic poate fi localizat n sistemul:

1. art.spinale anterioare
2. art.spinale posterioare
3. la nivelul zonelor critice
 Arteriovenous malformation

A B
Sagittal MR scans of the thoracic spinal cord: T2 fast spin-echo technique (A) and T1 post-contrast
image (B). On the T2-weighted image (left), abnormally high signal intensity is noted in the central
aspect of the spinal cord (arrowheads). Numerous punctate flow voids indent the dorsal and ventral
spinal cord (arrow). These represent the abnormally dilated venous plexus supplied by a dural
arteriovenous fistula. After contrast administration (B), multiple, serpentine, enhancing veins (arrows)
on the ventral and dorsal aspect of the thoracic spinal cord are visualized, diagnostic of arteriovenous
malformation. This patient was a 54-year-old man with a 4-year history of progressive paraparesis.
 MAV Spinal : de tip Ghem (Glomus
Type)

Rosenblumetal.,1987

ET
MAV Spinal : de tip Ghem (Glomus
Type) PE STNGA: Imagine
RMN sagital T2
ponderat
demonstreaz
creterea n volum a
mduvei spinrii, T2-
hiperintensitate
medular i vene
medulare intra- i
extramedulare lrgite.

CENTRU i PE
DREAPTA: Faza
arterial i cea care
urmeaz n angiografia
vertebral (proiecie
antero-posterioar)
demonstreaz artera
radiculomedular de
nivel C5 crescut n
dimensiuni (sgeata
roie) alimentnd un
MAV compact cu nidus
de tip ghem (sgeata
galben) graie ASA
fenestrat (sgeile
 MAV Spinal : Tip Juvenil

Rosenblumetal.,1987

RD
MIELOPATIA COMPRESIV
Compresia medular neoplazic

Regiunea toracic medular este de

cele mai dese ori implicat.

Durerea este semnul clinic de debut.

Examenul de elecie - RMN.

Epidural spinal cord compression due to breast
carcinoma

A B

Sagittal T1-weighted (A) and T2-weighted (B) MRI scans through

the cervicothoracic junction reveal an infiltrated and collapsed
second thoracic vertebral body with posterior displacement and
compression of the upper thoracic spinal cord. The low-intensity
bone marrow signal in A signifies replacement by tumor.
Treatment: NEOPLASTIC SPINAL CORD
COMPRESSION

Glucocorticoids to reduce cord edema

(dexamethasone, up to 40 mg daily).
Local radiotherapy (initiated as early as
possible) to the symptomatic lesion (generally
3000 cGy administered in 15 daily fractions) .
Specific therapy for the underlying tumor type.
 MRI of a thoracic meningioma

Coronal T1-weighted post- contrast image through the thoracic spinal cord
demonstrates intense and uniform enhancement of a well-circumscribed
extramedullary mass (arrows) which displaces the spinal cord to the left.
MRI of an intramedullary astrocytoma

Sagittal T1-weighted post-contrast image through the cervical spine

demonstrates expansion of the upper cervical spine by a mass lesion
emanating from within the spinal cord at the cervicomedullary junction.
Irregular peripheral enhancement occurs within the mass (arrows).
MRI of a spinal epidural abscess due to tuberculosis

A B

A. Sagittal T2-weighted free spin-echo MR sequence. A hypointense mass

replaces the posterior elements of C3 and extends epidurally to compress
the spinal cord (arrows). B. Sagittal T1-weighted image after contrast
administration reveals a diffuse enhancement of the epidural process
(arrows) with extension into the epidural space.
Treatment: SPINAL EPIDURAL
ABSCESS

decompressive laminectomy
with debridement
combined with long-term antibiotic treatment.
EVALUATION OF ACUTE TRANSVERSE
MYELOPATHY
1. MRI of spinal cord with and without contrast (exclude
compressive causes).
2. CSF studies: Cell count, protein, glucose, IgG index/synthesis rate,
oligoclonal bands, VDRL; Grams stain, acid-fast bacilli, and India ink
stains; PCR for VZV, HSV-2, HSV-1, EBV, CMV, HHV-6, enteroviruses, HIV;
antibody for HTLV-I, B. burgdorferi, M. pneumo- niae, 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. Also consider nasal/pharyn- geal/anal
cultures for enteroviruses; stool O&P for Schistosoma ova.
Note: VDRL, Venereal Disease Research Laboratory; PCR, polymerase chain reaction; VZV,
varicella-zoster virus; HHV, human herpes virus; RPR, rapid plasma reagin (test); O&P, ova
and parasites; ESR, erythrocyte sedimentation rate; ANA, antinuclear antibodies; ENA,
epithelial neutrophil-activating peptide.
EVALUATION OF ACUTE TRANSVERSE
MYELOPATHY
4. 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
syndrome suspected, Schirmer test, salivary gland scintography,
and salivary/lacrimal gland biopsy.
5. Sarcoidosis: Serum angiotensin-converting enzyme; serum Ca;
24-h urine Ca; chest x-ray; chest CT; total body gallium scan;
lymph node biopsy.
6. Demyelinating disease: Brain MRI scan, evoked potentials, CSF
oligoclonal bands, neuromyelitis optica antibody (aquaporin-4).
7. Vascular
Note: causes:Disease
VDRL, Venereal CT myelogram; spinal PCR,
Research Laboratory; angiogram.
polymerase chain reaction; VZV,
varicella-zoster virus; HHV, human herpes virus; RPR, rapid plasma reagin (test); O&P, ova
and parasites; ESR, erythrocyte sedimentation rate; ANA, antinuclear antibodies; ENA,
epithelial neutrophil-activating peptide.
 MRI of syringomyelia associated with a Chiari
malformation

Sagittal T1-weighted image through the cervical and upper

thoracic spine demonstrates descent of the cerebellar tonsils
and vermis below the level of the foramen magnum (black
arrows). Within the substance of the cervical and thoracic
spinal cord, a CSF collection dilates the central canal (white
arrows).
CHRONIC MYELOPATHIES
SUBACUTE COMBINED DEGENERATION (VITAMIN B12 DEFICIENCY

Treatable myelopathy.
Loss of vibration and position sensation, and a progressive spastic
and ataxic weakness.
Loss of reflexes due to an associated peripheral neuropathy in a
patient who also has Babinski signs.
Tends to be diffuse rather than focal. + Rombergs sign.
Macrocytic red blood cells, serum B12 concentration, serum
levels of homocysteine and methylmalonic acid, and in uncertain
cases a + Schilling test.
Treatment is by replacement therapy, beginning with 1000 g of
intramuscular vitamin B12 repeated at regular intervals or by
subsequent oral treatment.
CHRONIC MYELOPATHIES
HYPOCUPRIC MYELOPATHY

Is virtually identical to subacute combined degeneration with

normal serum levels of B12.
of serum copper and often there is also a of serum
ceruloplasmin.
Some cases follow gastrointestinal procedures that result in
impaired copper absorption, but many others are idiopathic.

Improvement or at least stabilization may be expected with

reconstitution of copper stores by oral supplementation.

The pathophysiology and pathology are not known.

REHABILITATION OF SPINAL CORD
DISORDERS

The prospects for recovery from an acute destructive spinal cord

lesion fade after ~6 months.
The disability associated with irreversible spinal cord damage is
determined primarily by the level of the lesion and by whether
the disturbance in function is complete or incomplete.
Even a complete high cervical cord lesion may be compatible
with a productive life.
The primary goals are development of a rehabilitation plan
framed by realistic expectations and attention to the neurologic,
medical, and psychological complications that commonly arise.
EXPECTED NEUROLOGIC FUNCTION
FOLLOWING COMPLETE CORD LESIONS
LEVEL SELF-CARE TRANSFERS MAXIMUM
MOBILITY
High Dependent on Dependent on Motorized
quadriplegia others; requires others wheelchair
(C1-C4) respiratory support
Low Partially May be May use manual
quadriplegia independent with dependent or wheelchair, drive
(C5-C8) adaptive equipment independent an automobile
with adaptive
equipment
Paraplegia Independent Independent Ambulates short
(below T1) distances with
aids
Source: Adapted from JF Ditunno, CS Formal: Chronic spinal
cord injury. N Engl J Med 330:550, 1994.
REHABILITATION OF SPINAL CORD
DISORDERS
Bladder dysfunction

Bladder dysfunction generally results from loss of supraspinal

innervation of the detrusor muscle of the bladder wall and the
sphincter musculature.
Detrusor spasticity is treated with anticholinergic drugs
(oxybutinin, 2.55 mg qid) or tricyclic antidepressants with
anticholinergic properties (imipramine, 25200 mg/d).
Failure of the sphincter muscle to relax during bladder emptying
(urinary dyssynergia) may be managed with the -adrenergic
blocking agent terazosin hydrochloride (12 mg tid or qid), with
intermittent catheterization, or, if that is not feasible, by use of a
condom catheter in men or a permanent indwelling catheter.
Surgical options.
REHABILITATION OF SPINAL CORD
DISORDERS
Risk for venous thrombosis and pulmonary embolism

During the first 2 weeks, use of calf-

compression devices and anticoagulation with
heparin (5000 U subcutaneously every 12 h) or
warfarin (INR, 23) are recommended.

In cases of persistent paralysis,

anticoagulation should probably be continued
for 3 months.
REHABILITATION OF SPINAL CORD
DISORDERS
Prophylaxis against decubitus ulcers

Frequent changes in position in a chair or bed, the use

of special mattresses, and cushioning of areas where
pressure sores often develop, such as the sacral
prominence and heels.

Early treatment of ulcers with careful cleansing,

surgical or enzyme debridement of necrotic tissue, and
appropriate dressing and drainage may prevent
infection of adjacent soft tissue or bone.
REHABILITATION OF SPINAL CORD
DISORDERS
Spasticity

Stretching exercises to maintain mobility of joints.

Baclofen (15240 mg/d in divided doses) is effective; it
acts by facilitating GABA-mediated inhibition of motor
reflex arcs.
Diazepam acts by a similar mechanism and is useful
for leg spasms that interrupt sleep (24 mg at
bedtime).
Tizanidine (28 mg tid), an 2 adrenergic agonist that
increases presynaptic inhibition of motor neurons, is
another option.
Intrathecal baclofen administered via an implanted
pump, botulinum toxin injections, or dorsal rhizotomy.
REHABILITATION OF SPINAL CORD
DISORDERS

Paroxysmal autonomic hyperreflexia.

Headache, flushing, and diaphoresis above the level of
the lesion.
Hypertension with bradycardia or tachycardia.
The trigger is typically a noxious stimulus - for
example, bladder or bowel distention, a urinary tract
infection, or a decubitus ulcer - below the level of the
cord lesion.
Treatment consists of removal of offending stimuli;
ganglionic blocking agents (mecamylamine, 2.55 mg)
or other short-acting antihypertensive drugs are useful
in some patients.
FURTHER READINGS

COLE JS, PATCHELL RA: Metastatic epidural spinal cord compression.

Lancet Neurol. 7:459, 2008
JACOB A, WEINSHENKER BG: An approach to the diagnosis of acute
transverse myelitis. Semin Neurol 28:95, 2008
KALB RG: Getting the spinal cord to think for itself. Arch Neurol 399
60:805, 2003
KRINGS T, GEIBPRASERT S: Spinal dural arteriovenous fistulas. AJNR: Am J
Neuroradiol. 30:639, 2009
KUMAR N: Copper deficiency myelopathy (human swayback). Mayo Clin
Proc 81:1371, 2006
TRANSVERSE MYELITIS CONSORTIUM WORKING GROUP: Proposed
diagnostic criteria and nosology of acute transverse myelitis. Neurology
59:499, 2002
TRAUL DE et al: Part I: Spinal-cord neoplasms - intradural neoplasms.
Lancet Oncol 8:35, 2007