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Color Atlas of Neurology

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Metastases

Spinal metastasis from bronchial carcinoma

 

 

Cranial metastasis

 

 

 

MRI (contrast-

 

Intradural/

 

 

 

 

 

 

 

enhanced, sagittal

 

leptomeningeal

 

 

 

T1-weighted image

 

metastasis

 

 

 

of thoracic spine)

 

Vertebral body metastasis

 

 

 

 

 

 

 

 

 

 

causing secondary spinal cord

Leptomeningeal metastasis

 

 

compression

 

 

Epidural metastasis

 

 

Cerebral

 

 

 

 

 

 

 

Metastatic

 

metastases

 

 

compression of vessel

 

 

 

 

 

(radicular a.)

 

 

 

 

 

Radicular

 

 

Spinal metastases

metastasis

 

 

 

 

 

 

Development of

 

 

 

 

 

 

 

 

Malignant cells

 

 

 

neoplasm distant from CNS

 

 

 

 

 

 

 

infiltrating veins and

 

 

 

 

 

 

 

lymph vessels

 

 

 

 

 

 

 

 

 

 

 

 

 

Systemic spread of

 

 

 

 

 

 

 

 

malignant cells

 

Systemic

 

 

 

 

 

 

(via foramen ovale)

 

spread of

 

 

 

 

Invasion of lung

malignant

 

 

 

 

 

cells

 

 

 

 

via pulmonary a.

 

 

 

 

 

 

 

 

Invasion of

 

right heart

 

by malignant

Malignant cells

cells

Patent foramen ovale

Pulmonary metastases

Pathogenesis of cerebral metastasis

Central Nervous System

263

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Brain Tumors

Classification and Treatment

 

As the treatment and prognosis of brain tumors

 

depend on their histological type and degree of

 

malignancy, the first step of management is

 

tissue diagnosis (see Table 31, p. 377). The sub-

 

sequent clinical course may differ from that pre-

 

dicted by the histological grade because of

 

“sampling error” (i.e., biopsy of an unrepresen-

 

tative portion of the tumor). Other factors in-

 

fluencing prognosis include age, the complete-

System

ness of surgical resection, the preoperative and

postoperative neurological findings, tumor pro-

gression, and the site of the tumor.

 

Nervous

! Incidence (adapted from Lantos et al., 1997)

The most common primary intracranial tumors

in patients under 20 years of age are medullo-

blastoma, pilocytic astrocytoma, ependymoma,

Central

and astrocytoma (WHO grade II); from age 20 to

age 45, astrocytoma (WHO grade II), oligoden-

 

 

droglioma, acoustic

neuroma

(schwannoma),

 

and ependymoma; over age 45, glioblastoma,

 

meningioma, acoustic neuroma, and oligoden-

 

droglioma. The overall incidence of pituitary

 

tumors (including

pituitary

metastases),

craniopharyngioma, and intracranial lymphoma and sarcoma is low.

! Severity (Table 32, p. 378)

The Karnofsky scale (Karnofsky et al., 1951) is a commonly used measure of neurological disability, e. g., due to a brain tumor. Its use permits a standardized assessment of clinical course.

! Treatment

The initial treatment is often neurosurgical, with the objective of removing the tumor as completely as possible without causing a severe or permanent neurological deficit. The resection can often be no more than subtotal because of the proximity of the tumor to eloquent brain areas or the lack of a distinct boundary between the tumor and the surrounding tissue. The overall treatment plan is usually a combination of different treatment modalities, chosen with consideration of the patient’s general condition and the location, extent, and degree of malignity

264of the tumor.

Symptomatic treatment. Edema: The an-

tiedematous action of glucocorticosteroids

takes effect several hours after they are administered; thus, acute intracranial hypertension must be treated with an intravenously given osmotic agent (20% mannitol). Glycerol can be given orally to lower the corticosteroid dose in chronic therapy. Antiepileptic drugs (e. g., phenytoin or carbamazepine) are indicated if the patient has already had one or more seizures, or else prophylactically in patients with rapidly growing tumors and in the acute postoperative setting. Pain often requires treatment (headache, painful neoplastic meningeosis, painful local tumor invasion; cf. WHO staged treatment scheme for cancer-related pain). Restlessness: treatment of cerebral edema, psychotropic drugs (levomepromazine, melperone, chlorprothixene). Antithrombotic prophylaxis: Subcutaneous heparin.

Grade I tumors. Some benign tumors, such as those discovered incidentally, can simply be ob- served—for example, with MRI scans repeated every 6 months—but most should be surgically resected, as a total resection is usually curative. Residual tumor after surgery can often be treated radiosurgically (if indicated by the histological diagnosis). Pituitary tumors and craniopharyngiomas can cause endocrine disturbances. Meningiomas and craniopharyngiomas rarely recur after (total) resection.

Grade II tumors. Five-year survival rate is 50–80%. Complete surgical resection of grade II tumors can be curative. As these tumors grow slowly, they are often less aggressively resected than malignant tumors, so as not to produce a neurological deficit (partial resection, later resection of regrown tumor if necessary). Observation with serial MRI rather than surgical resection may be an appropriate option in some patients after the diagnosis has been established by stereotactic biopsy; surgery and/or radiotherapy will be needed later in case of clinical or radiological progression. Chemotherapy is indicated for unresectable (or no longer resectable) tumors, or after failure of radiotherapy

Grade III tumors. Patients with grade III tumors survive a median of 2 years from the time of diagnosis with the best current treatment involving multiple modalities (surgery, radiotherapy, chemotherapy). Many patients, however, live considerably longer. There are still inadequate

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Brain Tumors

data on the potential efficacy of chemotherapy against malignant forms of meningioma, plexus papilloma, pineocytoma, schwannoma, hemangiopericytoma, and pituitary adenoma.

Grade IV tumors. Patients with grade IV tumors survive a median of ca. 10 months from diagnosis even with the best current multimodality treatment (surgery, radiotherapy, chemotherapy). The 5-year survival rate of patients with glioblastoma is no more than 5%. PNET (including medulloblastoma) and primary cerebral lymphoma have median survival times of a few years.

Cerebral metastases: Solitary, surgically accessible metastases are resected as long as there is no acute progression of the underlying malignant disease, or for tissue diagnosis if the primary tumor is of unknown type. Solitary metastases of diameter less than 3 cm can also be treated with local radiotherapy, in one of two forms: interstitial radiotherapy with surgically implanted radioactive material (brachytherapy), or stereotactic radiosurgery. The latter is a closed technique, requiring no incision, employing multiple radioactive cobalt sources (as in the Gamma Knife and X-Knife) or a linear accelerator. Solitary or multiple brain metastases in the setting of progressive primary disease are generally treated with whole-brain irradiation. Chemotherapy is indicated for tumors of known

responsiveness to chemotherapy in patients whose general condition is satisfactory. Metastatic small-cell lung cancer, primary CNS lymphoma, and germ cell tumors are treated with radiotherapy or chemotherapy rather than surgery.

Spinal metastases: Resection and radiotherapy for localized tumors; radiotherapy alone for diffuse metastatic disease.

Leptomeningeal metastases: Chemotherapy (systemic, intrathecal, or intraventricular); irradiation of neuraxis.

! Aftercare

Follow-up examinations are scheduled at shorter or longer intervals depending on the degree of malignity of the neoplasm and on the outcome of initial management (usually involving some combination of surgery, radiotherapy, and chemotherapy), with adjustment for individual factors and for any complications that may be encountered in the further course of the disease. A single CT or MRI scan 3 months postoperatively may suffice for the patient with a completely resected, benign tumor, while patients with malignant tumors should be followed up by examination every 6 weeks and neuroimaging every 3 months, at least initially. Later visits can be less frequent if the tumor does not recur.

Central Nervous System

265

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Central Nervous System

Trauma

Traumatic Brain Injury (TBI)

The outcome of traumatic brain injury depends on the type and extent of the acute (primary) injury and its secondary and late sequelae.

Direct/indirect history. A history of the precipitating event and of the patient’s condition at the scene should be obtained from the patient (if possible), or from an eyewitness, or both. Vomiting or an epileptic seizure in the acute aftermath of the event should be noted. Also important are the past medical history, current medications (particularly anticoagulants), and any history of alcoholism or drug abuse.

Physical examination. General: Open wounds, fractures, bruises, bleeding or clear discharge from the nose or ear. Neurological: Respiration, circulation, pupils, motor function, other focal signs.

Diagnostic studies. Laboratory: Blood count, coagulation, electrolytes, blood glucose, urea, creatinine, serum osmolality, blood alcohol, drug levels in urine, pregnancy testing if indicated.

Essential radiological studies: Head CT with brain and bone windows is mandatory in all cases un-

less the neurological examination is completely normal. A cervical spine series from C1 to C7 is needed to rule out associated cervical injury. Plain films of the skull are generally unnecessary if CT is performed.

Additional studies, as indicated: Cranial or spinal MRI or MR angiography, EEG, Doppler ultrasonography, evoked potentials.

In multiorgan trauma: Blood should be typed and cross-matched and several units should be kept ready for transfusion as needed. Physical examination and ancillary studies for any fractures, abdominal bleeding, pulmonary injury.

! Primary Injury

The primary injury affects different parts of the skull and brain depending on the precipitating event. The traumatic lesion may be focal (hematoma, contusion, infarct, localized edema) or diffuse (hypoxic injury, subarachnoid hemorrhage, generalized edema). The worse the injury, the more severe the impairment of consciousness (pp. 116 ff). The clinical assessment of impairment of consciousness is described on pp. 378 f (Tables 33 and 34).

Region

Type of Injury

 

 

Scalp

Cephalhematoma (neonates), laceration, scalping injury

Skull

" Fracture mechanism: Bending fracture (caused by blows to the head, etc.), burst frac-

 

 

ture (caused by broad skull compression)

 

" Fracture type: Linear fracture (fissure, fissured fracture, separation of cranial sutures),

 

 

impression fracture, fracture with multiple fragments, puncture fracture, growing

 

 

skull fracture (in children only)

 

" Fracture site: Convexity (calvaria), base of skull

 

" Basilar skull fracture: Frontobasal (bilateral periorbital hematoma (“raccoon sign”),

 

 

bleeding from nose/mouth, CSF rhinorrhea) or laterobasal (hearing loss, eardrum le-

 

 

sion, bleeding from the ear canal, CSF otorrhea, facial nerve palsy)

 

" Facial skull fracture: LeFort I–III midface fracture; orbital base fracture

Dura mater

Open head trauma1, CSF leak, pneumocephalus, pneumatocele

Blood vessels

Acute epidural, subdural, subarachnoid or intraparenchymal hemorrhage; carotid–

 

cavernous sinus fistula; arterial dissection

Brain2

"

Contusion

 

" Diffuse axonal injury (clinical features: coma, autonomic dysfunction, decortication

 

 

or decerebration, no focal lesion on CT or MRI)

 

" Penetrating (open or closed3) injury, or perforating (open) injury

 

"

Brainstem injury

 

 

 

1 Wound with open dura and exposure of brain (definition). 2 Excluding cranial nerve lesions. 3 Without dural penetration (definition).

266

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Trauma

 

 

 

Lacerations

 

 

 

 

 

 

Cranial impression

 

 

 

 

 

 

Depressed skull

 

 

 

 

 

 

 

 

 

fracture, hematoma,

 

 

 

dural opening

 

 

 

Gunshot

 

 

 

 

 

 

 

 

 

 

 

wound, hema-

 

 

 

toma along

 

 

 

trajectory

 

 

 

Brain herniation,

 

 

 

 

Head injuries

 

 

edema

 

 

 

 

 

 

 

Hemorrhagic contusion

 

Inner and outer dural layer

Inner

 

 

Outer

dural layer

 

 

 

 

dural layer

 

 

 

 

 

 

Epidural

 

 

 

hematoma

Traumatic intracranial hematoma

 

(Duration of

 

unconsciousness)

Moderate

 

24 h

HT

 

 

GCS:

Mild HT

9-12

1 h

 

GCS:

 

13-15

 

Head trauma (schematic)

Subdural hematoma

Retroauricular ecchymosis (due to basilar skull fracture)

Severe

HT

GCS: 3-8

Classification of head trauma (HT) by Glasgow Coma Scale (GCS)

Central Nervous System

267

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Central Nervous System

268

Trauma

! Secondary Sequelae of TBI

Type of Sequela

Location/Syndrome

Special Features

 

 

 

 

 

Neurological

 

 

 

 

"

Hematoma

Epidural

Lucid interval1, immediate uncon-

 

 

 

 

sciousness, or progressive deterioration of

 

 

 

 

consciousness

 

 

Subdural

May be asymptomatic at first, with

 

 

 

 

progressive decline of consciousness

 

 

Subarachnoid

Meningism

 

 

Intraparenchymal

Intracranial hypertension, focal signs;

 

 

 

 

often a severe injury

"

Intracranial

"

Cerebral edema, hydrocephalus,

"

See p. 162. Risk of herniation

 

hypertension

 

massive hematoma

 

 

"

Ischemia

" Vasospasm, arterial dissection, fat

"

Acute focal signs

 

 

 

embolism

 

 

"

Epileptic seizure

"

Focal or generalized

" Common in focal injury

"

Infection

"

CSF leak, open head injury

"

Recurrent meningitis, encephalitis,

 

 

 

 

 

empyema, abscess, ventriculitis

"

Amnesia

"

Anterograde/retrograde

"

See p. 134

General

 

 

 

 

"

Hypotension,

"

Shock, respiratory failure

" Multiple trauma, pneumothorax or

 

hypoxia, anemia

 

 

 

hemothorax, pericardial tamponade,

 

 

 

 

 

blood loss, coagulopathy

"

Fever,

"

Infection

"

Pneumonia, sepsis, CSF leak

 

meningitis

 

 

 

 

"

Fluid imbalance

"

Hypothalamic lesion

"

Diabetes insipidus2, SIADH3

1 Patient immediately loses consciousness awakens and appears normal for a few hours again loses consciousness. 2 Polyuria, polydipsia, nocturia, serum osmolality !295 mOsm/kg, urine osmolality. 3 Syndrome of inappropriate secretion of ADH: euvolemia, serum osmolality " 275 mOsm/kg, excessively concentrated urine (urine osmolality !100 mOsm/kg), urinary sodium despite normal salt/water intake; absence of adrenal, thyroid, pituitary and renal dysfunction.

For overview of late complications of head trauma, see p. 379 (Table 35).

! Prognosis

Head trauma causes physical impairment and behavioral abnormalities whose severity is correlated with that of the initial injury.

Severity1

Prognosis

 

 

Mild

Posttraumatic syndrome resolves within 1 year in 85–90% of patients. The re-

 

maining 10–15% develop a chronic posttraumatic syndrome

Moderate

The symptoms and signs resolve more slowly and less completely than those of

 

mild head injury. The prognosis appears to be worse for focal than for diffuse in-

 

juries. Reliable data on the long-term prognosis are not available

Severe

Age-dependent mortality ranges from 30% to 80%. Younger patients have a bet-

 

ter prognosis than older patients. Late behavioral changes (impairment of

 

memory and concentration, abnormal affect, personality changes)

 

 

1 For severity of head trauma, cf. pp. 378 f, Tables 33 and 34.

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Trauma

Cystic

Frontal brain

 

Brain atrophy, normal pressure hydrocephalus

 

 

 

postcon-

atrophy

 

(ventricular dilatation)

 

tusional

 

 

 

 

 

 

defect

 

 

 

 

 

 

 

 

 

Frontal sinus,

 

 

 

 

 

 

 

 

 

 

 

fracture

Basilar skull

 

 

 

 

 

 

fracture,

 

 

 

CSF

sphenoid

 

 

 

sinus

 

 

 

leak from

 

nose

Infection, abscess (penetrating injury)

 

 

Cerebral complications of trauma

 

CSF leak

CSF leak

(nasopharyngeal space)

Bilateral

 

 

Pneumocephalus

 

Infarct

 

 

Postcontu-

 

Frontal

 

 

 

 

chronic subdural

(air in intracranial

(posterior

sional lesion

brain atrophy

hematoma

cavity)

cerebral artery)

 

 

 

 

 

 

Posttraumatic neurological changes

 

Normal

Retrograde Trauma

Unconscious-

Anterograde

Normalization

memory

amnesia

ness or coma

amnesia

of memory

 

 

 

 

 

 

 

 

 

 

function

(Time)

Time course of memory disturbances

(closed head injury)

Central Nervous System

269

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Central Nervous System

270

Trauma

! Posttraumatic Headache

Intracerebral

hematoma.

Bleeding

into

the

tissue of the brain (intraparenchymal hema-

 

Posttraumatic headache may be acute (!8

toma) under the site of impact, on the opposite

weeks after head trauma) or chronic ("8

side (contre-coup), or in the ventricular system

weeks). The duration and intensity of the head-

(intraventricular hemorrhage) (pp. 176, 267).

ache are not correlated with the severity of the

Subarachnoid hemorrhage. Rupture of pial ves-

precipitating head trauma. It can be focal or dif-

sels.

 

 

 

 

 

 

 

fuse, continuous or episodic. It often worsens

! Treatment

 

 

 

 

 

 

with physical exertion, mental stress, and ten-

 

 

 

 

 

 

 

 

 

 

 

 

 

 

sion and improves with rest and stress

At the scene of the accident. The scene should

avoidance. Its type and extent are highly varia-

be secured to prevent further injury to the in-

ble. If the headache gradually increases in sever-

jured person, bystanders, or rescuers. First aid:

ity, or if a new neurological deficit arises, further

Evaluation and clearing of the airway; car-

studies should be performed to exclude a late

diopulmonary resuscitation (CPR) if necessary.

posttraumatic complication, such as chronic

Immobilization of the cervical spine with a hard

subdural hematoma (p. 379).

collar. Recognition and treatment of hemody-

! Pathogenesis of Traumatic Brain Injury

namic instability (keep systolic blood pressure

above

120 mmHg),

fluid

administration

as

 

Direct blunt or penetrating injuries of the head

needed (“small volume resuscitation” with hy-

and acceleration/deceleration injuries can dam-

peroncotic-hypertonic solutions’). Dressing of

age the scalp, skull, meninges, cerebral vascula-

wounds, sedation if necessary to reduce agita-

ture, ventricular system, and brain parenchyma.

tion, elevation of the upper body to 30°. Docu-

The term primary injury refers to the initial me-

mentation: Time and nature of accident, general

chanical damage to these tissues. Traumatized

and neurological findings, drugs given. Trans-

brain tissue is more sensitive to physiological

port: Cardiorespiratory monitoring.

 

 

changes than nontraumatized tissue. Secondary

In the hospital. Systematic assessment and

injury is caused by cellular dysfunction due to

treatment by organ system, with documenta-

focal or global changes in cerebral blood flow and

tion of all measures taken. Cardiorespiratory

metabolism. Mechanisms involved in secondary

monitoring: monitoring of blood gases and

injury include disruption of the blood–brain bar-

blood

pressure

(cerebral

perfusion

pressure

rier, hypoxia, neurochemical changes (increased

"60–70 mmHg,

p. 162).

Respiratory

system:

concentrations of acetylcholine, norepinephrine,

Supplementary oxygen, intubation, and ventila-

dopamine, epinephrine, magnesium, calcium,

tion as needed. Cardiovascular system: Central

and excitatory amino acids such as glutamate),

venous access, administration of fluids and

cytotoxic processes (production of free radicals

pressors as needed. Treatment of fever or hyper-

and of calcium-activated proteases and lipases),

thermia. Administration of anticonvulsants as

and inflammatory responses (edema, influx of

needed. Evaluation of tetanus vaccination sta-

leukocytes and macrophages, cytokine release).

tus. Immediate neurosurgical consultation re-

Epidural hematoma. Bleeding into the epidural

garding the possible need for surgery. Treatment

space (pp. 6, 267) due to detachment of the

of intracranial hypertension: Sedatives, analges-

outer dural sheath from the skull and rupture of

ics; if

ICP

(p. 162)

is above 20–25 mmHg,

a meningeal artery (usually the middle mening-

osmotherapy with 20% mannitol, bolus of

eal artery, torn by a linear fracture of the tem-

0.35 mg/kg over 10–15 minutes, repeated every

poral bone). Epidural hematoma is less

4–8 hours as needed; barbiturate coma

frequently of venous origin (usually due to tear-

(thiopental);

 

decompressive

bifrontal

ing of a venous sinus by a skull fracture).

craniectomy may be indicated in refractory

Subdural hematoma. Bleeding into the subdural

cerebral edema.

 

 

 

 

 

space (pp. 6, 176, 267) because of disruption of

 

 

 

 

 

 

 

 

larger bridging veins; often accompanied by

 

 

 

 

 

 

 

 

focal contusion of the underlying brain.

 

 

 

 

 

 

 

 

Frequently located in the temporal region.

 

 

 

 

 

 

 

 

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Trauma

Traumatic brain injury

Blood-brain barrier lesion

Hypoxia

Neurochemical changes

Cytotoxic processes

Inflammatory response

Posttraumatic headache

Hemorrhagic contusion

Pathogenesis of traumatic brain injury

 

 

 

 

 

 

 

 

Ensure that airways are free and unobstructed

 

 

 

 

Check cardiopulmonary function

 

 

 

 

 

 

 

 

Stable lateral position: Patient is

Supine position: Patient is unconscious, not

unconscious but breathing spontaneously

 

breathing (cardiopulmonary resuscitation),

 

 

and may have spinal injury. Elevate upper

 

 

body if there is a head injury

First-aid measures at scene of accident

Central Nervous System

271

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Central Nervous System

Trauma

Spinal Trauma

Spinal injury can involve the vertebrae, ligaments, intervertebral disks, blood vessels, muscles, nerve roots, and spinal cord. The spinal cord and spinal nerve roots may be directly injured (e. g. by gunshot or stab wounds) or secondarily affected by compression (bone fragments), hyperextension (spinal instability), and vascular lesions (ischemia, hemorrhage). Diagnosis: Bone injuries can be identified by radiography and/or CT; spinal cord lesions (hemorrhage, contusion, edema, transection) and softtissue lesions (hematoma, edema, arterial dissection) are best seen on MRI.

Cervical spine distortion (whiplash injury). Indirect spinal trauma (head-on or rear-end collision) leads to sudden passive retroflexion and subsequent anteflexion of the neck. The forces acting on the spine (acceleration, deceleration, rotation, traction) can produce both cervical spine injuries (spinal cord, nerve roots, retropharyngeal space, bones, ligaments, joints, intervertebral disks, blood vessels) and cranial injuries (brain, eyes, temporomandibular joint). There may be an interval of 4–48 hours until symptoms develop, rarely longer (asymptomatic period). Symptoms and signs: Pain in the head, neck, and shoulders, neck stiffness, and vertigo may be accompanied by forgetfulness, poor concentration, insomnia, and lethargy. The symptoms usually resolve within 3–12 months but persist for longer periods in 15–20% of patients, for unknown reasons. Severity classification: Grade I = no neurological deficit or radiological abnormality, grade II = neurological deficit without radiological abnormality, grade III = neurological deficit and radiological abnormality.

bral disks as composed of three columns. Involvement of only one column = stable injury; two columns = potentially unstable; three columns = unstable. For details, see p. 380 (Table 36).

Trauma to nerve roots and brachial plexus.

Nerve root lesions usually involve the ventral roots, and thus usually produce a motor rather than sensory deficit. Nerve root avulsion may be suspected on the basis of (multi)radicular findings and/or Horner syndrome and can be confirmed by myelography (empty root sleeves, bulging of the subarachnoid space) or MRI. Downward or backward traction on the shoulder and arm (as in a motorcycle accident) can produce severe brachial plexus injuries accompanied by nerve root avulsion. Brachial plexus lesions can also be caused by improper patient positioning during general anesthesia, intense supraclavicular pressure (backpack paralysis), or local trauma (stab or gunshot wound, bone fragments, contusion, avulsion). These injuries more commonly affect the upper portion of the brachial plexus (pp. 34, 321).

Vertebral fracture. It must be determined whether the fracture is stable or unstable; if it is unstable, any movement can cause (further) damage to the spinal cord and nerve roots. Thus, all patients who may have vertebral fractures must be transported in a stabilized supine position, with the head in a neutral position (e. g., on a vacuum mattress). Repositioning the patient manually with the “collar splint grip,” “paddle

272grip,” or “bridge grip” should be avoided if possible. In the assessment of stability, it is useful to consider the spinal column and interverte-

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