DEVELOPMENTAL MEDICINE AND CHILD NEUROLOGY. 1978, 20

Kugler, J., Knorl, G., Empt, J. (1973) ‘Die Behandlung therapieresistenter Epilipsien mit Dipropylacetat.’
Miinrhener Medizinische Wochenschrift, 115, 1103-1 119.
Voss, H. von, Petrich, C., Karch, D., Schulz, H. U., Gobel, U. (1976) ‘Sodium valproate and platelet
function.’ British Medicul Journal, 2, 179.
Winfield, D. A., Benton, P., Espir, M. L., Arthur, L. J. (1976) ‘Sodium valproate and thronibocytopenia.’
British Medical Journal, 2,981.

Cortical Blindness Associated with Occipital Atrophy:
a Complication of H. injuenzae Meningitis
Lewis H. Margolis

Bennett A . Shaywitz

Introduction
Cortical blindness is a rare and generally
unexplained complication of bacterial
meningitis. This communication reports a
case of persistent cortical blindness secondary to Hemophilus injluenzae type-b
meningitis with an associated anatomical
lesion in the vicinity of the visual cortex, as
determined by computerized axial tomography (CT scan). To our knowledge this
is the first description of an associated
anatomical abnormality in a child with
cortical blindness secondary to bacterial
meningitis.

Stephen G. Rothman

locytes. Within minutes after the lumbar puncture
he suffered a respiratory arrest but was ventilated
by ‘Ambu’ bag within seconds and was soon intubated. Antibiotic therapy was begun with chloraniphenicol (lWmg/kg/day) and penicillin G
(300,000U/kg/day).
Symptoms resolved rapidly and within 24 hours
his behaviour became more normal. 48 hours after
admission penicillin and chloramphenicol were discontinued and ampicillin (400mg/kg/day) was begun. Four days after admission he developed focal
seizures of the right arm and it became clear that
his eyes did not follow light. A repeat lumbar
puncture indicated non-xanthochromic fluid with
an opening pressure of 200mm H,O, CSF glucose
64mg/dl, protein 25mg/dI, 1590 red blood cells,
and 1080 total nucleated cells, 90 per cent granulocytes. An electroencephalogram demonstrated
moderate generalized slowing, most pronounced
in the left temporal region. A Technetium brainscan revealed increased bilateral occipital activity,
consistent with subdural empyema or meningeal
inflammation. CT scan performed 14 days after
admission (Fig. 1 ) was consistent with hemorrhagic
infarction in the occipital area. At the time of discharge the patient continued to exhibit evidence o f
cortical blindness, as well as minor lower extremity
weakness.
Computerized tomography performed 2+
months after his illness demonstrated bilateral
occipital atrophy (Fig. 2). In the six months since
his discharge there has been no other neurological
abnormality apart from persisting cortical blindness: his intellectual development appears to remain normal.

Case Report
G.A., a three-year-old white male was well until
24 hours prior to admission, when he developed a
temperature of 39’C. He was examined at a local
hospital and sent home, but later developed a
temperature of 40.6’C. He was then admitted to
the local hospital and transferred to Yale-New
Haven Hospital for evaluation. During the
journey he was given a dose of intravenous mannitol (1g;kg) because of a dilated right pupil. On
admission he was stuporous and had marked
nuchal rigidity. The optic discs were normal
bilaterally and the pupils were equally reactive. He
did not seem to follow light, but this was difficult
to assess because of his stuporous level of consciousness. Examination of the CSF indicated an
opening pressure of 300mm H,O, CSF glucose
13mg d l , protein 107mg’dl, 1380 red blood cells
and 1300 total nucleated cells. 98 per cent granu-

Discussion
Cortical blindness, characterized by loss
of vision with normal pupillary response,
has a number of reported causes in chil-

Cor.rc~.~p:pondence
to Bennett A. Shaywitz, M.D.,
Yale University School of Medicine, 333 Cedar
Street, New Haven, Connecticut 06510.

490

CASE REPORTS

dren. McAuley (1964) reported a child of
2 years 7 months who suffered a cardiorespiratory arrest and hypothermia after
falling through ice. Early in his treatment
this child exhibited cortical blindness
associated with an abnormal EEG, characterized by “flattening and irregular delta
waves”, which returned to regular 5-6/sec
activity in the occipital area as he regained
vision within about eight weeks. Weinberger et al. (1962) described three children
aged between 24 and 3 years who developed temporary cortical blindness following cardiac arrest.
Griffith and Dodge (1968) described six

children with transient cortical blindness
secondary to head trauma. In two,
electroencephalograms demonstrated bilateral occipital slowing, which resolved,
but the authors emphasize the uncertain
significance of this finding because posterior slowing frequently follows head
trauma in children.
Acers and Cooper (1965) described
cortical blindness secondary to bacterial
meningitis in two children. One child, four
months old, failed to respond to light or
other visual stimuli five days after the onset
of H. influenzae meningitis, but had
apparently normal vision one month later.

Fig. 1. Serial CT sections through the superior portion of the brain, demonstrating diffuse high density
throughout the cortical sulci.

Fig. 2. Serial scans done 10 weeks after the acute illness demonstrate symmetrical dilatation of the occipital
pole, suggesting localized occipital atrophy.

49 1

DEVELOPMENTAL MEDICINEAND CHILD NEUROLOGY. 1978, 20

gitis in a three-year-old boy. A Technetium
scan demonstrated increased uptake in the
occipital area, consistent with an effusion
or inflammation. 1 I days later, computerized tomography showed a serpiginous
high-density structure corresponding to
the cortical gyral pattern and thought to
be cortical hemorrhagic infarction. One
mechanism to explain the hemorrhage is
transtentorial herniation of the temporal
lobes, leading to transient compression of
the posterior cerebral arteries. The dilated
pupil, the high opening CSF pressure, the
presence of 1380 red blood cells, and
the respiratory arrest support this possibility. However, if the hemorrhage had
occurred secondary to herniation, we
would have expected the CT scan to
demonstrate more resolution of blood two
weeks after the presumed event. This
suggests that the hemorrhage occurred at
a later time in the illness. Another possible
mechanism is that venous occlusion,
arteritis or thrombophlebitis may have
produced the hemorrhage. Finally, it
is possible that the initial effusion on
Technetium scan was a thick exudate
which resulted in cerebral necrosis, but
the clinical course is not consistent with a
significant subdural effusion. When the
blindness persisted, a repeat CT scan 10
weeks later demonstrated symmetrical
dilatation of the occipital poles, suggesting
localized occipital atrophy.
The persisting blindness six months
later and the established atrophy demonstrated by computerized tomography make
it unlikely that this child will recover his
vision.

The second, a five-month-old with pneumococcal meningitis, was still blind at
follow-up seven months after the illness.
Barnet ef ul. (1970) examined visual
responses (VERS) in six children with
cortical blindness and found depressed
responses in three patients who remained
blind, but normal or near normal VERS in
two children who soon regained their
vision. Tn a subsequent study, however,
Duchowny et al. (1974) suggested that
the VERS did not aid in determining a
prognosis for the return of vision, although
there was correlation between specific
VER findings and the concurrent neurological status. They described four cases with
proven bacterial meningitis, and in three
of these the complication was temporary.
The first child, a 16;-month-old with H.
injhenzae, had minimal residual blindness
one month after her illness. The second
was a 26-month-old girl with meningococcus who recovered from her blindness
about six weeks after the meningitis. A
16-month-old boy with H. infruenzae
recovered normal vision by the fifteenth
day of illness. However, the fourth child,
a 7k-month-old girl, remained blind and
was severely intellectually retarded following pneumococcal meningitis.
The mechanism of cortical blindness
associated with meningitis remains illdefined. Pathologically, subarachnoid exudate, arteritis, focal necrosis of veins,
thrombophlebitis, subdural exudate and
temporary venous occlusion have all been
described and may relate to the pathogenesis of cortical damage (Adams et al.
1948, Smith and Landing 1965). Dodge
and Swartz (1965) emphasized that cortical
damage can only in part be explained by
anatomical findings and that a ‘toxic
factor’, aspiration, shock, fever and increased CSF pressure may all contribute to
brain damage.
We have described a case of cortical
blindness following H. infruenxe menin-

Acknowledgement: This study was supported in
part by a grant from The National Institute of
Health (NS 12384).
AUTHORS’ APPOINTMENTS

Lewis H. Margolis, M.D., Instructorin Pediatrics;
Bennett A. Shaywitz, M.D., Associate Professor
of Pediatrics and Neurology;
Stephen G. Rothman, M.D., Associate Professor
of Radiology;
Yale University School of Medicine. New Haven,
Connecticut 06510.

492

CASE REPORTS

SUMMARY

A three-year-old child with H . influenzae meningitis developed cortical blindness. Computerized tomography demonstrated occipital hemorrhage, and a subsequent scan showed
occipital atrophy. The literature on cortical blindness in children is reviewed. This is the
first description of cortical blindness secondary to meningitis with an associated anatomical
defect.
RESUME

Ctcitt corticale associte a une atrophie occipitale:
complication d‘une mtningite 2 Himophilus influenzae
Un garGon de trois ans, atteint de mkningite 8 H. influenzae, prtsenta une ckcitk corticale.
Une tomo-densitographie montra des hkmorragies occipitales et un scanner ultirieur
rkvtla une atrophie occipitale. I1 s’agit 18 de la premikre description d’une ckcitt corticale
secondaire a une mtningite avec altiration anatomique associke.
ZUSAMMENFASSUNG

Corticale Blindheit mit occipitaler Atrophie:
eine Kornlpikation der H . influenza Meningitis
Bei einem dreijahrigen Jungen trat nach einer H. influenza Meningitis eine corticale
Blindheit auf. Die Komputertomographie zeigte eine occipitale Blutung und spatere Kontrolluntersuchungen ergaben eine occipitale Atrophie. Dies ist die erste Beschreibung einer
corticalen Blindheit nach Meningitis n i t einem anatomischen Defekt.
RESUMEN

Ceguera cortical asociada con atrojia occipital:
una complicacidrz de la meningitis par H . H. influenzae
Un niiio de tres aiios de edad con meningitis por H. H. influenzae desarroll6 una ceguera
cortical. La TAC demostr6 una hemorragia cerebral y 10s Scanners subsiguientes mostracon
una atrofia occipital. Esta es la primera descripci6n de ceguera secundaria a meningitis con
un defect0 anat6mico asociado.
REFERENCES
Acers, T. E., Coopcr, W. C. (1965) ‘Cortical blindness secondary to bacterial meningitis.’ American Journal
of Ophthalmology, 59, 226-229.
Adams, R. D., Kubik, C. S., Bonner, F. J. (1948) ‘Clinical and pathological aspects of influenzal meningitis.’
Archives of Pediatrics, 65,354316; 408-441.
Barnet, A. B., Manson, J. I., Wilner, E, (1970) ‘Acute cerebral blindness in children,’ Neurology, 20, 11471156.
Dodge, P. R., Swartz, M. N. (1965) ‘Bacterial meningitis-a review of selected aspects. 11. Special neurologic
problems, postmeningitis complications and clinico-pathological correlations.’ New England Journal of
Medicine, 272, 1003-1010.
Duchowny, M. S., Weiss, I. P., Majlessi, H., Barnet, A. B. (1974) ‘Visual evoked responses in childhood
cortical blindness after head trauma and meninigitis.’ Neurology, 24,933-940.
Griffith, J. F., Dodge, P. R. (1968) ‘Transient blindness following head injury in children.’ New England
Journal of Medicine, 278,648-651.
McAuley, F. D. (1964) ‘Prolonged visual failure following cardiac arrest due to accidental hypothennia.’
British Journal of Ophthalmology, 48,628-629.
Smith, J. F., Landing, B. H. (1965) ‘The clinical and pathological aspects of influenzal meningitis.’ Journal
of Neuropathology and Experimental Neurology, 19,248-265.
Weinberger, H. A., van der Woude, R., Maier, H. C. (1962) ‘Prognosis of cortical blindness following cardiac
arrest in children.’Journal of the American Medical Association, 179,126-129.

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