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Ataxia after severe head injury The pathological substrate.

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BRIEF COMMUNICATIONS
Ataxia after
Severe Head Injury:
The Pathological Substrate
C. Susan Chester, MD,"? and Barry R. Reznick, M D t
Ataxia is a common finding in patients who have recovered from severe head injury. This report delineates
the clinical and pathological findings in a patient who
recovered from a head injury but was left with ataxia of
gait. A lesion of the superior cerebellar peduncle, demonstrated post mortem 2% years after injury, was the
only explanation for the ataxia. This lesion is part of the
spectrum of changes seen in diffuse axonal injury
thought to be due to shearing forces which tear white
matter at the time of the initial injury.
Chester CS, Reznick BR: Ataxia after severe
head injury: the pathological substrate.
Ann Neurol22:77-79, 1987
Clinicopathological studies of head in juries have generally been limited to patients who died soon after
injury or to those dying after prolonged coma. Unexplained focal deficits are common in patients who survive a serious injury, but the pathological correlates of
the focal lesions are not well defined.
This report describes a patient who recovered from
a head injury, but was left with ataxia and died 2'/2
years later. Demyelination of the superior cerebellar
peduncle, demonstrated post mortem, was the only
explanation for the ataxia. The occurrence of lesions in
the cerebellar peduncle and corpus callosum after
trauma is well known, but this is usually associated
with a prolonged vegetative state.
Case Report
A 32-year-old salesman was found unconscious with
Cheyne-Stokes respirations after his car hit a telephone pole.
Subsequent examiners described fixed, dilated pupils and
withdrawal to painful stimuli. S i x hours later, decerebrate
posturing of the right limbs developed. A skull film demonstrated a right parietal fracture. Computed tomography
showed the lateral ventricles were displaced to the right by a
large hematoma within the left temporal lobe as well as contusions in the right parietal lobe. At surgery, the left tempo-
From the * D e p m e n t of Neurology, Case Western Reserve University School of Medicine, and +Department of Neuropathology
and Neurology, Cleveland Metropolitan General Hospital, Cleveland, OH.
Received Aug 5 , 1786, and in revised form Nov 7. Accepted for
publication Nov 7, 1786.
Address correspondence to Dr Chester, Cleveland Metropolitan
General Hospital, 3395 Scranton Rd, Cleveland, OH 44109.
ral intracerebral hematoma and a left-sided subdural hematoma were aspirated.
After surgery, intracerebral pressure was 5 to 10 mm of
cerebrospinal fluid. He was unresponsive for 10 days, but
gradual improvement occurred over the next 12 weeks. He
was transferred to a rehabilitation unit where subsequent
examination revealed dysarthric speech with paraphasic errors and no understanding of oral or written directions. Despite adequate strength, he could not stand without assistance. Even when sitting unsupported, he would fall. If he
walked with others supporting him, he lifted both legs high
and put them down in an irregular manner. Mild horizontal
nystagmus occurred with left lateral gaze. There was bilateral
ataxia on finger-to-nose and heel-to-shin maneuvers. There
was slight hyperreflexia on the left and an extensor plantar
response on the right.
His comprehension and speech gradually improved during
an 11-week hospitalization, although his speech remained
dysarthric with many paraphasic errors. His judgment was
poor and he lost his temper easily. Six months after the
accident, he was able to stand and walk with a walker, although he would easily topple over backwards. His gait remained ataxic and a moderate dysmetria persisted. Further
examinations revealed no deficit in proprioception or perception of pinprick, touch, and vibration. He was able to do
simple tasks in the yard and house but was not able to return
to work. Two and a half years after the automobile accident,
he died by drowning.
Pathology
Inspection of the brain revealed the hallmarks of an
old traumatic brain injury. Numerous firm yellow
plaques were present over the frontal lobes (particularly on the left), the right superior temporal gyrus,
and the suprasylvian areas of the parietal lobe. These
discolored regions were limited to the cortex and were
maximal at the crests of the gyri. A single smaller lesion in the right inferior parietal gyrus extended into
the white matter. The left temporal lobe was the site of
tissue necrosis and cavitation. Only the tip of the temporal lobe, hippocampus, and amygdala showed no
changes. Multiple small streaks, which were concentrated on the left of the midline, discolored the genu of
the corpus callosum. The ventricular system was not
enlatged, and the brainstem and cerebellum were
grossly normal.
The gray matter of the cerebral hemispheres had
patchy areas with loss of neurons, gemistocytic astrocytes, and hemosiderin-laden macrophages. In the left
temporal lobe, there was complete loss of normal architecture with irregular cysts extending deep into
white matter. The cysts were surrounded by an astrocytic reaction and hemosiderin-laden macrophages.
Two lesions in the white matter were demonstrated
by the use of myelin stains. The first lesion was in the
anterior part of the corpus callosum. The second was
in the left superior cerebellar peduncle (brachium conjunctivum) as it traveled through the pons and mid77
Fig 1. In the midbrain, loss of myeiinatedfibers in the brachium
conjunctivum a t the Iwel of the red nucleus accentuates the intact
fibers of the left oculomotor nerve (arrows). (Myelin stain; R =
right; L = left.,
Fig 2. Comparison of the sides in this section of pons reveals a
loss of myeIinatedfibers in the left brachium conjuncthwm
(arrow). (Myelin stain; R = right; L = It$.)
brain. This lesion began near the junction of the cerebellar peduncle and brainstem. It did not extend to the
cerebellar nuclei. In the midbrain, the demyelination
continued through the decussation of brachium conjunctivum but not into the thalamus (Figs 1, 2). Bodian
stains demonstrated disruption and loss of axons
within the brachium conjunctivum and corpus callosum. The cellular reaction was limited to a subtle
microcystic change with reactive astrocytes in the
superior cerebellar peduncle. In the corpus callosum,
an astrocytic reaction, perivascular cuffing of mononuclear cells, and some hemosiderin-laden macrophages remained.
78 Annals of Neurology Vol 22 No 1 July 1987
Discussion
This case represents one of the few autopsy studies of
a patient with a severe traumatic brain injury and prolonged coma who regained consciousness and achieved
a moderate degree of recovery. Autopsy demonstrated
the pathological changes that are usually found in patients who die soon after the trauma or who remain in
intractable coma. Thiese changes, namely necrosis in
one or both superior cerebellar peduncles and the corpus callosum, are the hallmarks of the abnormal
changes attributed to primary injury to axons at the
time of the impact [l, 2, 5, 6, 9, 11, 12, 14).
Clinical and pathological studies have clearly delineated the features 'of white matter lesions resulting
from trauma or diffuse axonal injury. The patients are
unconscious with decerebrate posturing from the time
of accident, and the coma is protracted and unrelenting. The incidence of skull fractures is low compared
to that in patients who do not have white matter lesions. Few have intracranial hematomas. Intracranial
pressure is elevated in only 56% of patients with diffuse axonal in jury. Pathological studies have confirmed
the stereotyped nature of the lesions and the progression from mild hemorrhage and necrosis to the development of glial reaction and cystic cavities. Often the
lesion in the cerebellar peduncle is unilateral. There is
usually also striking evidence of trauma in the cerebral
hemispheres, primarily contusion [ 1, 2). An experimental model developed by Gennarelli and colleagues
171, which used angular acceleration in primates, has
duplicated both of these pathological changes and a
clinical spectrum of unconsciousness similar to that observed in traumatized humans.
Only two other reports have demonstrated white
matter lesions in patients who have regained consciousness and achieved any notable degree of recovery following trauma [12, 13). Although the clinical
information about these patients is limited, both
showed ataxia or an unspecified gait disorder. Ataxia,
often unexplained, is a. common sequela of head
trauma. Gilchrist and Wilkinson have reported that
54% of 84 patients with head injury showed ataxia of
limbs and dysarthria [8). In other series of patients
completing rehabilitation programs, 8 to 33% of the
patients were ataxic [4, 8, lo). Anoxia has often been
invoked as the cause of thie cerebellar findings in these
patients 131.
On the basis of the pathological changes in our patient and the others sumniarited above, it is apparent
that another cause of ataxia in this population is damage to the white matter in the superior cerebellar
peduncle, which is peculiarly vulnerable to rotational
injury because of its anatomical location.
Furthermore, it is apparent that the focal white matter lesions in the brainstern and corpus callosum may
appear without extensive demyelination within the cerebral hemispheres. Axonal injury caused by trauma is
both a focal and diffuse process, and there is a spectrum of destruction, the severity of which determines
some of the focal deficits in survivors of head trauma.
Because the damage to the superior cerebellar peduncle is a subtle finding, demonstrated best by myelin
stains, such lesions can easily go undetected. Appropriate pathological evaluation of patients who have suffered closed head injuries mandates careful evaluation
of the cerebellar peduncles.
We are grateful for the encouragement and support of Dr Betty Q.
Banker.
References
1. Adams JH, Graham DI, Murray LS, Scott G: Diffuse axonal
injury due to nonmissile head injury in humans: an analysis of
45 cases. Ann Neurol 12:557-563, 1982
2. Adams JH, Mitchell DE, Graham DI, Doyle D Diffuse brain
damage of immediate impact type: its relationship to primacy
brainstem damage in head injury. Brain 100:489-502, 1977
3. Adams RD, Victor M V Principles of Neurology. New York,
McGraw-Hill, 1985, pp 641-664
4. Brink JD, Imbus C, Woo-Sam J: Physical recovery after severe
closed head trauma in children and adolescents. J Pediatr
97:721-727, 1980
5. Crompton M R Brainstem lesions due to closed head injury.
Lancet 1:669-673, 1971
6. Crompton MR, Teare RD, Bowen DAL Prolonged coma after
head injury. Lancet 1:938-940, 1966
7. Gennarelli TA, Thibault LE, Adams JH, et al: Diffuse axonal
injury and traumatic coma in the primate. Ann Neurol 12:564574, 1982
8. Gilchrist E, Wilkinson M: Some factors determining prognosis
in young people with severe head injuries. Arch Neurol
36355-359, 1979
9. Jellinger K, Seitelberger F Protracted post-traumatic encephalopathy: pathology, pathogenesis and clinical implications. J
Neurol Sci 10:51-94, 1970
10. Roberts AH: Long term prognosis of severe accidental head
injury. Proc R SOCMed 69:137-140, 1976
11. Strich SJ: Diffuse degeneration of the cerebral white matter in
severe dementia following head injury. J Neurol Neurosurg
Psychiatry 19:I63-185, 1956
12. Strich SJ, Oxon DM: Shearing of nerve fibers as a cause of brain
damage due to head injury: a pathological study of twenty cases.
Lancet 2:443-448, 1961
13. T o d n s o n BE: Brain-stem lesions after head injury. J Clin
Pathol [Suppll 4:154-165, 1970
14. Van der Zwan A: Late results from prolonged traumatic unconsciousness. In Walker AE, Caveness WF, Critchley MD (eds):
The Late Effects of Head Injury. Springfield, IL, Thomas, 1969,
pp 138-149
Giant Axonal Neuropathv:
.
Correlation of Clinic&
Findings with Postmortem
Neuropathology
C. Thomas, MD,X S. Love, MB, BCh, PhD,*
H. C. Powell, MD,' P. Schultz, MD,?
and P. W. Lampert, MD"
We report the clinical and postmortem neuropathological findings in a case of long-standing giant axonal neuropathy. The patient, a Caucasian male with kinky hair,
was first seen at 4 years of age because of increasing
unsteadiness of gait. Clinical examination showed nystagmus, cerebellar ataxia, distal sensory loss, and weakness. A surd nerve biopsy at 8 years of age revealed
giant axonal neuropathy. The patient became increasingly demented and was incapacitated by weakness and
ataxia; he died at 18 years of age. Histological examination of the brain and spinal cord showed numerous Rosenthal fibers, a distal axonopathy that most severely
affected the corticospinal tracts, middle cerebellar
peduncles, and posterior columns, and olivocerebellar
degeneration.
Thomas C, Love S, Powell HC, Schultz P, Lampert PW:
Giant axonal neuropathy: correlation of clinical
findings with postmortem neuropathology.
Ann Neurol 22:79-84, 1987
In 1972, Asbury and colleagues 111 described a
progressive sensorimotor polyneuropathy, associated
with axonal distention by neurofilaments, which they
termed giant axonal neutvpathy (GAN). Approximately twenty cases have been reported since (see for
example [b, 111). Ultrastructural and immunohistochemical studies have shown aggregation of intermediate filaments in many cell types, including Schwann
cells, endothelial cells, perineurial cells, and fibroblasts
16 111.
Most cases of GAN have clinical or electrophysiological evidence of central nervous system (CNS) dysfunction. Patients often present with cerebellar ataxia
From the "Department of Pathology (M-O12D), University of
California, San Diego, School of Medicine, La Jolla, CA 92093, and
?Department of Pediatrics, Children's Hospital, 8001 Frost St, San
Diego, CA 92123.
Received July 15, 1986, and in revised form Nov 4. Accepted for
publication Nov 7, 1986.
Address correspondence to Dr Love, Department of Pathology
(M-O12D), University of California, San Diego School of Medicine,
La JolIa, CA 92093.
79
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