St Louis Encephalitis With Particular Involvement of the Brain Stem Allen M.
Kaplan, MD, Jules
T.
Koveleski,
MD
of St Louis encephalitis in a 4-year-old child in which the main clinical features were dominated by brain stem dysfunction. We document an drome and present a review. We also discuss St Louis encephalitis, with special attention to the ocular manifestations. \s=b\ We
report a
case
(Arch Neurol 35:45-46, 1978)
The diffuse
neurologic manifestations of St Louis encephalitis (SLE) are
and have been well docu¬ mented.14 Although pathologic inves¬ tigations of SLE cases have described profound microscopic brain stem changes, clinical signs referable to brain stem dysfunction are only infre¬ quently reported. These include vari¬ ous cranial nerve palsies, nystagmus, tremor, ataxia, and alterations in sensorium. This communication reports a case of SLE in a 4-year-old girl, with par¬ ticular brain stem involvement and an interesting transient Parinaud-like
syndrome.
REPORT OF A CASE A
4-year-old girl was admitted to the hospital in the late summer with a five-day history of malaise, fever, irritability, and anorexia. On the day of admission, the child was observed to have a grand mal
seizure of five minutes' duration. There was no history of craniocerebral trauma, drug, or toxic ingestion. The child had had frequent febrile seizures but was not given anticonvulsant therapy. Examination on admission showed an illappearing child with the following vital signs: blood pressure, 110/68 mm Hg; pulse, 64 beats per minute, respirations, 20/min, and temperature, 38.9 C. The general phys¬ ical examination was normal. The patient was responsive to deep pain and had spon¬ taneous respirations. There was obvious right-sided facial weakness (Fig 1). Initial
Accepted for publication July 18, 1977. From the Department of Pediatric Neurology, Good Samaritan Hospital, Phoenix, Ariz. Reprint requests to Department of Pediatric Neurology, Good Samaritan Hospital, 1033 E McDowell Rd, Phoenix, AZ 85006 (Dr Kaplan).
examination showed persistent tonic verti¬ cal gaze deviation downward (Fig 2), with absence of vertical oculocephalic reflexes and Bell's phenomenon. The pupils were 3 to 4 mm bilaterally and sluggishly reactive to light. Horizontal oculocephalic reflexes were normal. The funduscopic examination disclosed no evidence of papilledema or hemorrhages, and lower brain stem cranial nerve function was intact. The tone was generally increased, and decerebrate pos¬ turing was intermittently observed. The muscle stretch reflexes were increased, with clonus in the lower extremities. Babinski signs were present.
Laboratory studies, including complete blood count, urinalysis, serum electrolyte count, and blood chemistry studies were
normal except for mild elevations of cre¬ atine phosphokinase and lactic dehydrogenase levels. Roentgenograms of the chest and skull were normal. A lumbar puncture showed hazy CSF. The CSF pres¬ sure was not recorded. The WBC count was 440/cu mm, with 84% lymphocytes and 16% polymorphonuclear leukocytes. There were no RBCs. The CSF glucose value was 63 mg/100 ml and total protein content was 84 mg/100 ml. Cerebrospinal fluid and blood cultures were negative for bacteria, viruses, fungi, and tuberculosis. Titers for St Louis encephalitis demonstrated signifi¬ cant elevations from the acute to convales¬ cent serums, but other viral antibody titers were negative (Table). An EEG showed generalized high voltage 2- to 3-cps slowing without focal abnormality, and no epileptiform activity was noted.
Fig 1.—Facial weakness, with flattening of right nasolabial fold and poor lid closure.
Downloaded From: http://archneur.jamanetwork.com/ by a University of Pennsylvania User on 06/13/2015
On admission, the patient was given antibiotics that included chloramphenicol (100 mg/kg/day) and ampicillin sodium
(300 mg/kg/day) intravenously, pending culture results. Therapy was discontinued after 48 hours. During the following five days there was gradual improvement of the patient's sensorium, and the child became more responsive and would follow simple commands. The tonic downward deviation of the eyes persisted for 48 hours. The facial paresis present on admission also resolved within five days. Subsequent examination has shown a persistent neuro¬ logic deficit characterized by truncal and extremital ataxia, and dysarthric speech. The child is otherwise physically and intel¬ lectually normal. COMMENT
St Louis encephalitis was first recognized in epidemic proportions in Paris, 111 in 1932. Since then, numer¬ ous outbreaks have occurred through¬ out the country, usually in the late summer or early fall. Over 540 cases of SLE were confirmed in 1975, and an additional 157 cases have been re¬ ported in 1976.5 A classification of the various clinical presentations has been established and includes the follow¬ ing: (1) encephalitis, (2) aseptic menin¬ gitis, (3) febrile illness with headache, and (4) nonspecific illness.3 The mor¬ tality of SLE in children is low, but neurologic residua have been reported in as many as 54%.2 These sequelae are most commonly manifest as behavior problems, disturbance of gait and equilibrium, disorders of speech, per-
Fig
2.—Tonic
(downward) vertical gaze.
Serum Viral
Antibody
Agent Herpes simplex (CF)* Adenovirus (CF) St Louis encephalitis (CF) (HI) California encephalitis (CF) Eastern equine encephalitis (CF) Western equine encephalitis (CF) Venezuelan encephalitis (CF) Lymphocytic choriomeningitis (CF) Coccidioidomycosis (CF) Coxsackie 1-6 (CF) Viral
'CF denotes complement-fixation
conjunctival inflammation, unequal pupils, diplopia, blurring of vision, nystagmus, ptosis, papilledema, and exophthalmus.1413 Our patient dem¬ onstrated a prominent and inter¬ esting Parinaud-like syndrome char¬ acterized by tonic (downward) vertical gaze associated with absent vertical oculocephalic responses and Bell's phe¬
well
reactions.
as
sluggish pupillary
Clinicopathologic studies of vertical
eye movements1415 have indicated
that anatomic
pathways subserving upward gaze are located bilaterally in the brain stem, with probable decussation in the posterior commissure. Therefore, upward gaze paralysis may occur
Acute Sera 1:8 16
Kaplan, MD, Jules
T.
Koveleski,
MD
of St Louis encephalitis in a 4-year-old child in which the main clinical features were dominated by brain stem dysfunction. We document an drome and present a review. We also discuss St Louis encephalitis, with special attention to the ocular manifestations. \s=b\ We
report a
case
(Arch Neurol 35:45-46, 1978)
The diffuse
neurologic manifestations of St Louis encephalitis (SLE) are
and have been well docu¬ mented.14 Although pathologic inves¬ tigations of SLE cases have described profound microscopic brain stem changes, clinical signs referable to brain stem dysfunction are only infre¬ quently reported. These include vari¬ ous cranial nerve palsies, nystagmus, tremor, ataxia, and alterations in sensorium. This communication reports a case of SLE in a 4-year-old girl, with par¬ ticular brain stem involvement and an interesting transient Parinaud-like
syndrome.
REPORT OF A CASE A
4-year-old girl was admitted to the hospital in the late summer with a five-day history of malaise, fever, irritability, and anorexia. On the day of admission, the child was observed to have a grand mal
seizure of five minutes' duration. There was no history of craniocerebral trauma, drug, or toxic ingestion. The child had had frequent febrile seizures but was not given anticonvulsant therapy. Examination on admission showed an illappearing child with the following vital signs: blood pressure, 110/68 mm Hg; pulse, 64 beats per minute, respirations, 20/min, and temperature, 38.9 C. The general phys¬ ical examination was normal. The patient was responsive to deep pain and had spon¬ taneous respirations. There was obvious right-sided facial weakness (Fig 1). Initial
Accepted for publication July 18, 1977. From the Department of Pediatric Neurology, Good Samaritan Hospital, Phoenix, Ariz. Reprint requests to Department of Pediatric Neurology, Good Samaritan Hospital, 1033 E McDowell Rd, Phoenix, AZ 85006 (Dr Kaplan).
examination showed persistent tonic verti¬ cal gaze deviation downward (Fig 2), with absence of vertical oculocephalic reflexes and Bell's phenomenon. The pupils were 3 to 4 mm bilaterally and sluggishly reactive to light. Horizontal oculocephalic reflexes were normal. The funduscopic examination disclosed no evidence of papilledema or hemorrhages, and lower brain stem cranial nerve function was intact. The tone was generally increased, and decerebrate pos¬ turing was intermittently observed. The muscle stretch reflexes were increased, with clonus in the lower extremities. Babinski signs were present.
Laboratory studies, including complete blood count, urinalysis, serum electrolyte count, and blood chemistry studies were
normal except for mild elevations of cre¬ atine phosphokinase and lactic dehydrogenase levels. Roentgenograms of the chest and skull were normal. A lumbar puncture showed hazy CSF. The CSF pres¬ sure was not recorded. The WBC count was 440/cu mm, with 84% lymphocytes and 16% polymorphonuclear leukocytes. There were no RBCs. The CSF glucose value was 63 mg/100 ml and total protein content was 84 mg/100 ml. Cerebrospinal fluid and blood cultures were negative for bacteria, viruses, fungi, and tuberculosis. Titers for St Louis encephalitis demonstrated signifi¬ cant elevations from the acute to convales¬ cent serums, but other viral antibody titers were negative (Table). An EEG showed generalized high voltage 2- to 3-cps slowing without focal abnormality, and no epileptiform activity was noted.
Fig 1.—Facial weakness, with flattening of right nasolabial fold and poor lid closure.
Downloaded From: http://archneur.jamanetwork.com/ by a University of Pennsylvania User on 06/13/2015
On admission, the patient was given antibiotics that included chloramphenicol (100 mg/kg/day) and ampicillin sodium
(300 mg/kg/day) intravenously, pending culture results. Therapy was discontinued after 48 hours. During the following five days there was gradual improvement of the patient's sensorium, and the child became more responsive and would follow simple commands. The tonic downward deviation of the eyes persisted for 48 hours. The facial paresis present on admission also resolved within five days. Subsequent examination has shown a persistent neuro¬ logic deficit characterized by truncal and extremital ataxia, and dysarthric speech. The child is otherwise physically and intel¬ lectually normal. COMMENT
St Louis encephalitis was first recognized in epidemic proportions in Paris, 111 in 1932. Since then, numer¬ ous outbreaks have occurred through¬ out the country, usually in the late summer or early fall. Over 540 cases of SLE were confirmed in 1975, and an additional 157 cases have been re¬ ported in 1976.5 A classification of the various clinical presentations has been established and includes the follow¬ ing: (1) encephalitis, (2) aseptic menin¬ gitis, (3) febrile illness with headache, and (4) nonspecific illness.3 The mor¬ tality of SLE in children is low, but neurologic residua have been reported in as many as 54%.2 These sequelae are most commonly manifest as behavior problems, disturbance of gait and equilibrium, disorders of speech, per-
Fig
2.—Tonic
(downward) vertical gaze.
Serum Viral
Antibody
Agent Herpes simplex (CF)* Adenovirus (CF) St Louis encephalitis (CF) (HI) California encephalitis (CF) Eastern equine encephalitis (CF) Western equine encephalitis (CF) Venezuelan encephalitis (CF) Lymphocytic choriomeningitis (CF) Coccidioidomycosis (CF) Coxsackie 1-6 (CF) Viral
'CF denotes complement-fixation
conjunctival inflammation, unequal pupils, diplopia, blurring of vision, nystagmus, ptosis, papilledema, and exophthalmus.1413 Our patient dem¬ onstrated a prominent and inter¬ esting Parinaud-like syndrome char¬ acterized by tonic (downward) vertical gaze associated with absent vertical oculocephalic responses and Bell's phe¬
well
reactions.
as
sluggish pupillary
Clinicopathologic studies of vertical
eye movements1415 have indicated
that anatomic
pathways subserving upward gaze are located bilaterally in the brain stem, with probable decussation in the posterior commissure. Therefore, upward gaze paralysis may occur
Acute Sera 1:8 16
