
- •Overview
- •Preface
- •Translator’s Note
- •Contents
- •1. Fundamentals
- •Microscopic Anatomy of the Nervous System
- •Elements of Neurophysiology
- •Elements of Neurogenetics
- •General Genetics
- •Neurogenetics
- •Genetic Counseling
- •2. The Clinical Interview in Neurology
- •General Principles of History Taking
- •Special Aspects of History Taking
- •3. The Neurological Examination
- •Basic Principles of the Neurological Examination
- •Stance and Gait
- •Examination of the Head and Cranial Nerves
- •Head and Cervical Spine
- •Cranial Nerves
- •Examination of the Upper Limbs
- •Motor Function and Coordination
- •Muscle Tone and Strength
- •Reflexes
- •Sensation
- •Examination of the Trunk
- •Examination of the Lower Limbs
- •Coordination and Strength
- •Reflexes
- •Sensation
- •Examination of the Autonomic Nervous System
- •Neurologically Relevant Aspects of the General Physical Examination
- •Neuropsychological and Psychiatric Examination
- •Psychopathological Findings
- •Neuropsychological Examination
- •Special Considerations in the Neurological Examination of Infants and Young Children
- •Reflexes
- •4. Ancillary Tests in Neurology
- •Fundamentals
- •Imaging Studies
- •Conventional Skeletal Radiographs
- •Computed Tomography (CT)
- •Magnetic Resonance Imaging (MRI)
- •Angiography with Radiological Contrast Media
- •Myelography and Radiculography
- •Electrophysiological Studies
- •Fundamentals
- •Electroencephalography (EEG)
- •Evoked potentials
- •Electromyography
- •Electroneurography
- •Other Electrophysiological Studies
- •Ultrasonography
- •Other Ancillary Studies
- •Cerebrospinal Fluid Studies
- •Tissue Biopsies
- •Perimetry
- •5. Topical Diagnosis and Differential Diagnosis of Neurological Syndromes
- •Fundamentals
- •Muscle Weakness and Other Motor Disturbances
- •Sensory Disturbances
- •Anatomical Substrate of Sensation
- •Disturbances of Consciousness
- •Dysfunction of Specific Areas of the Brain
- •Thalamic Syndromes
- •Brainstem Syndromes
- •Cerebellar Syndromes
- •6. Diseases of the Brain and Meninges
- •Congenital and Perinatally Acquired Diseases of the Brain
- •Fundamentals
- •Special Clinical Forms
- •Traumatic Brain injury
- •Fundamentals
- •Traumatic Hematomas
- •Complications of Traumatic Brain Injury
- •Intracranial Pressure and Brain Tumors
- •Intracranial Pressure
- •Brain Tumors
- •Cerebral Ischemia
- •Nontraumatic Intracranial Hemorrhage
- •Infectious Diseases of the Brain and Meninges
- •Infections Mainly Involving the Meninges
- •Infections Mainly Involving the Brain
- •Intracranial Abscesses
- •Congenital Metabolic Disorders
- •Acquired Metabolic Disorders
- •Diseases of the Basal Ganglia
- •Fundamentals
- •Diseases Causing Hyperkinesia
- •Other Types of Involuntary Movement
- •Cerebellar Diseases
- •Dementing Diseases
- •The Dementia Syndrome
- •Vascular Dementia
- •7. Diseases of the Spinal Cord
- •Anatomical Fundamentals
- •The Main Spinal Cord Syndromes and Their Anatomical Localization
- •Spinal Cord Trauma
- •Spinal Cord Compression
- •Spinal Cord Tumors
- •Myelopathy Due to Cervical Spondylosis
- •Circulatory Disorders of the Spinal Cord
- •Blood Supply of the Spinal Cord
- •Arterial Hypoperfusion
- •Impaired Venous Drainage
- •Infectious and Inflammatory Diseases of the Spinal Cord
- •Syringomyelia and Syringobulbia
- •Diseases Mainly Affecting the Long Tracts of the Spinal Cord
- •Diseases of the Anterior Horns
- •8. Multiple Sclerosis and Other Myelinopathies
- •Fundamentals
- •Myelin
- •Multiple Sclerosis
- •Other Demyelinating Diseases of Unknown Pathogenesis
- •9. Epilepsy and Its Differential Diagnosis
- •Types of Epilepsy
- •Classification of the Epilepsies
- •Generalized Seizures
- •Partial (Focal) Seizures
- •Status Epilepticus
- •Episodic Neurological Disturbances of Nonepileptic Origin
- •Episodic Disturbances with Transient Loss of Consciousness and Falling
- •Episodic Loss of Consciousness without Falling
- •Episodic Movement Disorders without Loss of Consciousness
- •10. Polyradiculopathy and Polyneuropathy
- •Fundamentals
- •Polyradiculitis
- •Cranial Polyradiculitis
- •Polyradiculitis of the Cauda Equina
- •Polyneuropathy
- •Fundamentals
- •11. Diseases of the Cranial Nerves
- •Fundamentals
- •Disturbances of Smell (Olfactory Nerve)
- •Neurological Disturbances of Vision (Optic Nerve)
- •Visual Field Defects
- •Impairment of Visual Acuity
- •Pathological Findings of the Optic Disc
- •Disturbances of Ocular and Pupillary Motility
- •Fundamentals of Eye Movements
- •Oculomotor Disturbances
- •Supranuclear Oculomotor Disturbances
- •Lesions of the Nerves to the Eye Muscles and Their Brainstem Nuclei
- •Ptosis
- •Pupillary Disturbances
- •Lesions of the Trigeminal Nerve
- •Lesions of the Facial Nerve
- •Disturbances of Hearing and Balance; Vertigo
- •Neurological Disturbances of Hearing
- •Disequilibrium and Vertigo
- •The Lower Cranial Nerves
- •Accessory Nerve Palsy
- •Hypoglossal Nerve Palsy
- •Multiple Cranial Nerve Deficits
- •12. Diseases of the Spinal Nerve Roots and Peripheral Nerves
- •Fundamentals
- •Spinal Radicular Syndromes
- •Peripheral Nerve Lesions
- •Fundamentals
- •Diseases of the Brachial Plexus
- •Diseases of the Nerves of the Trunk
- •13. Painful Syndromes
- •Fundamentals
- •Painful Syndromes of the Head And Neck
- •IHS Classification of Headache
- •Approach to the Patient with Headache
- •Migraine
- •Cluster Headache
- •Tension-type Headache
- •Rare Varieties of Primary headache
- •Symptomatic Headache
- •Painful Syndromes of the Face
- •Dangerous Types of Headache
- •“Genuine” Neuralgias in the Face
- •Painful Shoulder−Arm Syndromes (SAS)
- •Neurogenic Arm Pain
- •Vasogenic Arm Pain
- •“Arm Pain of Overuse”
- •Other Types of Arm Pain
- •Pain in the Trunk and Back
- •Thoracic and Abdominal Wall Pain
- •Back Pain
- •Groin Pain
- •Leg Pain
- •Pseudoradicular Pain
- •14. Diseases of Muscle (Myopathies)
- •Structure and Function of Muscle
- •General Symptomatology, Evaluation, and Classification of Muscle Diseases
- •Muscular Dystrophies
- •Autosomal Muscular Dystrophies
- •Myotonic Syndromes and Periodic Paralysis Syndromes
- •Rarer Types of Muscular Dystrophy
- •Diseases Mainly Causing Myotonia
- •Metabolic Myopathies
- •Acute Rhabdomyolysis
- •Mitochondrial Encephalomyopathies
- •Myositis
- •Other Diseases Affecting Muscle
- •Myopathies Due to Systemic Disease
- •Congenital Myopathies
- •Disturbances of Neuromuscular Transmission−Myasthenic Syndromes
- •15. Diseases of the Autonomic Nervous System
- •Anatomy
- •Normal and Pathological Function of the Autonomic Nervous System
- •Sweating
- •Bladder, Bowel, and Sexual Function
- •Generalized Autonomic Dysfunction
- •Index

|
|
|
Traumatic Brain injury |
87 |
Table 6.5 Types of craniosynostosis (Continued) |
|
|
|
|
Type |
Fused suture |
Shape of head |
Remarks |
|
plagiocephaly |
premature fusion (or incomplete |
asymmetrical (e.g., flattened |
|
|
fusion) of a coronal suture |
on one side) |
|
|
|
|
often due to |
|
||
|
|
|
|
|
|
|
|
asymmetrical |
|
|
|
|
muscle tone in |
|
|
|
|
cerebral palsy |
|
Crouzon disease |
mainly the coronal |
(craniofacial |
suture, and maxillary |
dysostosis) |
sutures in the face |
trigonocephaly |
metopic suture |
broad skull and face, jutting |
the airway |
|
forehead, exophtalmos, |
||
may be |
||
hypertelorism, hook nose, |
||
compromised |
||
prognathism |
||
|
||
pointed forehead |
|
platycephaly |
lambdoid suture |
broad occiput |
Traumatic Brain injury
Fundamentals
Traumatic injuries of the bony skull and the underlying brain can be of different types and varying severity, depending on the nature and intensity of the causative event. There may be a skull fracture affecting the cranial vault or the base of the skull, a brain contusion, an injury to larger sized blood vessels producing a traumatic hematoma, or any combination of these types of injury.
Brain injuries are either closed (i. e., with the dura mater intact) or open (with a wound extending into the subdural compartment or deeper into the brain parenchyma). Open brain injuries are associated with the risk of early or late intracranial infection.
The scale of clinical severity of traumatic brain injury extends from a simple contusion of the bony skull to the concussion syndrome and the brain contusion syndrome. The leading clinical manifestation of a traumatic parenchymal injury is impairment or loss of consciousness, usually accompanied by memory impairment (retrograde and anterograde amnesia). A neurological deficit or epileptic seizures may also be present.
Large traumatic hematomas, or extensive damage to the brain parenchyma with accompanying edema, may lead to a rapid rise of intracranial pressure, caus-
blubber blubber
ing brain compression and possibly brainstem herniation.
Traumatic hematomas may be located within the brain parenchyma (traumatic intracerebral hematoma) or in the adjacent meningeal compartments (subdural and epidural hematoma). Traumatic subarachnoid hemorrhage is less common.
Frequent late complications of severe traumatic brain injury include neuropsychological deficits, personality changes, and symptomatic epilepsy.
Relevant Aspects of the Clinical History
and Neurological Examination
In the initial phase after trauma, the severity of injury is assessed, with particular attention to the following aspects of the history:
the duration of unconsciousness (as reported by eyewitnesses);
the duration of amnesia for events that occurred before the injury (retrograde amnesia);
the duration of amnesia for events that have occurred since the injury (anterograde amnesia, perhaps accompanied by confusion);
the duration of the entire period of amnesia, which is the sum of the durations of retroand anterograde amnesia;
Mumenthaler / Mattle, Fundamentals of Neurology © 2006 Thieme All rights reserved. Usage subject to terms and conditions of license.
6
Diseases of the Brain and Meninges

88 6 |
Diseases of the Brain and Meninges |
|
|||
|
|
|
|
|
|
|
|
early epileptic seizures; |
extensive skull fracture, but no neurological deficit; |
||
|
|
bleeding from the ear or nose (indicating a basilar |
another patient may sustain a relatively minor blow to |
||
|
|
skull fracture). |
the head that ruptures a bridging vein and produces a |
||
|
The depth of impaired consciousness or coma in a brain- |
slowly growing subdural hematoma, which can com- |
|||
|
press the brain and ultimately cause coma and death. The |
||||
injured patient is graded numerically on the Glasgow |
clinical state of the patient after traumatic brain injury |
||||
|
Coma Scale (Table 6.6). |
can be classified as follows (in order of increasing sever- |
|||
|
|
Important aspects of the initial physical examination |
ity): |
||
|
are: |
|
|||
|
the patient’s level of consciousness (see above); |
Skull contusion. Patients with the simple skull contu- |
|||
|
externally visible injuries, especially of the head; |
sion syndrome have no evidence of a brain injury, i. e., no |
|||
|
bleeding and possibly flow of cerebrospinal fluid from |
loss of consciousness or amnesia, a normal neurological |
|||
|
|
the nose or ears, or in the pharynx (a CSF leak is con- |
examination, and normal intracranial findings on CT or |
||
|
|
clusive evidence of an open brain injury, while bleed- |
MRI (if performed). Some patients with this syndrome |
||
|
|
ing is not); |
have scalp lacerations, or even skull fractures, and head- |
||
|
injuries of the cervical spine; |
ache may be present. Adequate therapy consists of a tem- |
|||
|
|
periorbital hematoma; neurological findings (pupil- |
porary restriction of activity and symptomatic medica- |
||
|
|
lary reflexes, visual impairment, nystagmus, deaf- |
tion, as required (analgesics, antiemetics). |
||
|
|
ness, weakness, pyramidal tract signs), general state |
Concussion (= mild traumatic brain injury) is character- |
||
|
|
of health, and, in particular, circulatory status. |
|||
|
Ancillary tests, to be performed as indicated by the |
ized by a brief, transient loss of consciousness, usually last- |
|||
|
ing no more than a few minutes and sometimes followed |
||||
|
clinical situation, include cervical spine radiographs and |
by a period of confusion. The periods of retroand antero- |
|||
|
a CT and/or MRI scan of the head. Skull radiographs are |
grade amnesia are very brief. Headache, dizziness, nausea, |
|||
hardly ever indicated. |
and (sometimes) vomiting are common accompani- |
||||
|
|
|
|
ments of concussion in the early phase. A standard neu- |
|
|
|
|
|
||
|
|
Traumatic brain injuries are often accompanied by |
rological examination reveals no deficit. In the past, it |
||
! cervical spine injuries. |
was generally assumed that concussion produced no |
||||
|
|
|
|
structural damage to the brain, but T2-weighted MR im- |
|
|
|
|
|
||
|
|
|
|
ages do, in fact, reveal diffuse axonal injury in some |
|
|
Grades of Severity of Traumatic |
patients. Moreover, neuropsychological testing reveals |
|||
|
that some patients said to have sustained no more than a |
||||
Brain Injury |
|||||
concussion actually have deficits of certain characteristic |
|||||
|
|
|
|
types, collectively designated minimal brain injury. Oc- |
|
|
The clinical grade of severity of traumatic brain injury is |
casional patients suffer from persistent posttraumatic |
|||
|
closely correlated with the initially evident extent of |
headaches. The clinical distinction between concussion |
|||
structural damage to the skull and brain, but the correla- |
and brain contusion (see below) is not always easy to |
||||
|
tion is not absolute. For example, a patient may have an |
draw. |
Table 6.6 The Glasgow Coma Scale
Category |
Points |
|
|
|
|
Best verbal response: |
|
|
none |
1 |
|
unintelligible sounds |
2 |
|
inappropriate words |
3 |
|
disoriented |
4 |
|
oriented |
5 |
|
Eye opening: |
|
|
none |
1 |
|
to painful stimuli |
2 |
|
to auditory stimuli |
3 |
|
spontaneous |
4 |
|
Best motor response: |
|
|
none |
1 |
|
abnormal extension |
2 |
|
abnormal flexion |
3 |
|
withdraws (pulls away from pain) |
4 |
|
localizes (fends off painful stimulus) |
5 |
|
follows commands |
6 |
|
|
|
|
Patient’s overall score |
. . . |
|
|
|
|
The overall score is the sum of the scores in the three categories.
Treatment of concussion. As in the skull contusion syndrome, transient restriction of activity suffices (e. g., a few days of bed rest), combined with symptomatic medication as required (see above). The patient should on no account be immobilized any longer than necessary: as long as there is no contraindication (such as hemodynamic instability), the patient should stand up and walk with assistance on the day of injury, or within the next few days at latest. Rapidly mobilized patients tend to have less severe postconcussive symptoms with a lesser tendency toward chronification.
Brain contusion and penetrating injuries. By definition, these types of injury involve damage to the brain parenchyma. Compared with concussion, they produce considerably longer periods of unconsciousness and retroand anterograde amnesia; indeed, the patient may not remember anything for a period of several days surrounding the time of the injury. Examination in the acute phase often reveals neurological deficits, which may persist. Residual anosmia is common (p. 180). CT or MRI reveals foci of contusion (Fig. 6.2) or intracranial hemorrhage, e. g., an acute epidural hematoma. Parenchymal injuries can be found both directly underlying the site of the external blow (“coup” injuries) and at the diametri-
Mumenthaler / Mattle, Fundamentals of Neurology © 2006 Thieme All rights reserved. Usage subject to terms and conditions of license.

Traumatic Brain injury
cally opposite location in the brain (“contrecoup” injuries). Injuries of the latter type are due to the violent, tissue-distorting force transmitted across the brain to the other side at the moment of injury. The Pathoanatomical findings in foci of brain contusion include ischemic and hemorrhagic tissue necrosis, small hemorrhages, tears of brain tissue and blood vessels, and secondary brain edema. Lumbar puncture, if performed (generally contraindicated!), yields bloody or xanthochromic cereobrospinal fluid.
|
Large brain contusions and extensive traumatic hema- |
|
||
tomas (see below), combined with the associated sec- |
|
|||
ondary brain edema, can cause very rapid and pro- |
|
|||
nounced increases in intracranial pressure, leading to |
|
|||
brain compression and herniation of the midbrain and |
|
|||
diencephalon through the tentorial notch, and/or of the |
|
|||
medulla through the foramen magnum. The clinical |
|
|||
signs of brainstem herniation are: progressive impair- |
Fig. 6.2 Brain contusion (CT scan). There are extensive hemor- |
|||
ment of consciousness leading to coma; a dilated pupil, |
||||
rhagic contusions in both temporal lobes and smaller ones in both |
||||
initially only on the side of the expansive lesion; flexor |
||||
frontal lobes (arrowheads). |
||||
and, later, extensor spasms; and, finally, a loss of auton- |
|
|||
omic regulatory functions (breathing, temperature, car- |
|
|||
diac activity, vascular tone), bilaterally fixed and dilated |
taken, including elevation of the patient’s head, hyper- |
|||
pupils, and death. |
||||
ventilation (in some patients), osmotherapy, or even |
||||
|
|
|
||
|
All patients with traumatic brain injury must be care- |
craniectomy to relieve brain compression (p. 93). Recent |
||
|
studies have shown a positive effect of therapeutic hy- |
|||
! fully clinically observed for signs of increasing in- |
||||
pothermia, in which the patient is cooled to ca. 34 °C. |
||||
|
tracranial pressure. In patients with a diminished level |
If the patient survives, MRI may reveal a permanent in- |
||
|
of consciousness, or coma, the width and reactivity of |
|||
|
jury to the brain parenchyma (Fig. 6.3). The late posttrau- |
|||
|
the pupils and other brainstem reflexes should be |
|||
|
matic symptoms resemble those of concussion, but they |
|||
|
checked regularly, so that intracranial hypertension |
|||
|
are more intense and usually persist longer. For further |
|||
|
can be detected at the earliest possible moment. In some |
|||
|
details, see p. 91. |
|||
|
patients, pressure-measuring devices will be implanted |
|||
|
|
|||
|
intracranially for continuous, invasive ICP monitoring. |
|
Treatment of the brain contusion syndrome. Depending on his or her clinical state, the patient may need to be observed in an intensive care unit or dedicated neurotrauma unit, with frequent checking of the vital signs and neurological functions, and possibly with invasive ICP monitoring. Extensive parenchymal injuries and the associated brain edema usually elevate the intracranial pressure; thus, ICP-reducing measures may need to be
Traumatic Hematomas
Traumatic hematomas come about when the traumatic injury tears a larger artery or vein (Fig. 6.4). They are classified as follows:
Intracerebral hematomas are usually found in the frontal or temporal lobe. They may exert considerable mass effect; combined with the surrounding edema,
89
6
Diseases of the Brain and Meninges
Fig. 6.3 Parenchymal defects
6 years after brain contusion.
The T2-weighted MR images reveal cortical defects in the left temporal (a) and frontal lobes (b), accompanied by signal changes in the underlying white matter.
a |
b |
blubber blubber
Mumenthaler / Mattle, Fundamentals of Neurology © 2006 Thieme All rights reserved. Usage subject to terms and conditions of license.

90 6 Diseases of the Brain and Meninges
they may cause sufficient pressure on the brain to produce a progressive decline of consciousness and increasingly severe neurological deficits. In such patients, neurosurgical evacuation of the hematoma should be considered, depending on its size and location.
Epidural hematomas (Fig. 6.5) are generally produced by traumatic tearing of a dural artery, usually the middle meningeal a. The tear itself is usually the result of a temporoparietal skull fracture, but sometimes occurs in the absence of a skull fracture. The blood collection lies between the periosteum and the dura mater. The arterial hemorrhage can compress the brain very rapidly: a patient who is initially comatose because of a coexisting brain contusion may fail to emerge from coma because of the development of an epidural hematoma in the minutes or hours after injury. On the other hand, an initially awake or only transiently unconscious patient may lapse into coma after a so-called “lucid interval” lasting minutes or hours. The side of the hematoma can often be ascertained by clinical examination: incipient uncal herniation compresses the ipsilateral oculomotor n. and causes dilation of the ipsilateral pupil, while the hemiparesis is contralateral to the hematoma. When an acute epidural hematoma is suspected, a CT scan should be performed immediately to confirm the diagnosis (not
Fig. 6.5 Epidural hematoma (CT scan).
plain films or MRI; see note, below). The hematoma is usually seen as a hyperdense, biconvex zone that is sharply demarcated from the adjacent brain tissue. Once diagnosed, it must be neurosurgically evacuated immediately to prevent brainstem herniation and death. Patients often make an excellent recovery if they
skull |
intracerebral hematoma |
acute subdural hematoma |
|
|
|
epidural space |
|
|
dura mater |
|
|
subdural space |
|
|
arachnoid |
|
|
subarachnoid space |
|
|
brain |
|
|
chronic subdural hematoma |
epidural hematoma |
CSF fistula |
rhinogenic posttraumatic meningo- |
malresorptive hydrocephalus |
posttraumatic focal epilepsy |
encephalitis |
|
|
Fig. 6.4 Traumatic brain injuries and posttraumatic complications (schematic diagram).
Mumenthaler / Mattle, Fundamentals of Neurology © 2006 Thieme All rights reserved. Usage subject to terms and conditions of license.