A case of reversible corpus callosum pressure lesion syndrome in children

Reversible corpus callosum pressure lesion syndrome (RESLES) is a new clinical and imaging syndrome proposed by Garcia-Monco et al.
in 2011, which is clinically manifested as symptoms of encephalitis or encephalopathy, and imaging tests detect reversible lesions
of corpus callosum pressure.
RESLES is rare in clinical practice, and more than 100 cases have been reported abroad, mostly in children
.
At present, there are no reports of cases of childhood RESLES in China
.
Recently, our hospital admitted a child
with RESLES caused by a central nervous system virus infection.
The clinical and imaging manifestations are summarized and analyzed, and the clinical and imaging characteristics
of RESLES in children are discussed in combination with relevant literature.

1 Clinical data

Child, male, 7 years old
.
He was admitted to hospital
with "fever and headache for 5 days".
The child developed fever and headache 5 days ago, with a temperature of up to 39.
7°C, with chills, no cough, abdominal pain, vomiting and convulsions
.
In other hospitals, there was no improvement after "ceftriaxone, ribavirin" and other drugs, and he was transferred to our hospital
.

The child has been in good health and has no other medical history
.
There is no special
family history.

Physical examination: clear consciousness, T 37.
4 °C, pulse 86 7 times/min, breathing 20 times/min, blood pressure 121/75 mmHg (1 mmHg = 0.
133kPa), weight 33 kg
.
pharyngeal hyperemia, cervical resistance (+), Brudzinski sign, Kernig sign (+); Other no abnormalities
.

Laboratory tests: blood, urine, stool routine, liver and kidney function is normal, blood sodium 127mmol/L, potassium, chloride is normal
.
Lumbar puncture CSF examination: WBC 480×106/L, lymphocytes 86%, monocytes 14%, protein 1.
42 g/L, sugar 2.
61 mmol/L, chloride 117 mmoL/L; Culture is negative
.
ECG, chest x-ray, and head CT are normal
.

Diagnosis of "viral meningitis"
.

Ganciclovir, mannitol, normal saline, and dexamethasone were treated with intravenous dexamethasone, and fever and headache
remained 4 days (8 days of onset) after hospitalization.
normal EEG; blood procalcitonin 0.
5 ng/ml, Epstein-Barr virus antibody (VCA—lgG)(+); Other common viruses and pathogen antibodies are all (one).

MRI of the head showed oval long T1 and long T2 signals in the corpus callosum pressure, and Flair and DWI hyperintensity shadows (Figure 1).

CSF was re-examined on the 5th day of hospitalization: WBC330×106/L, lymphocytes 68%, monocytes 32%, protein 0.
519/L, sugar 3.
46 mmoL/L, chloride 114 mol/L
.
Blood electrolytes: potassium 4.
7 mmol/L, sodium 129 mmol/L, chloride 94 mmol/L
.
The child's condition improved significantly, with fever, headache, and neck resistance disappearing
.

Fig.
1 The 9th day M block examination of the child showed that the oval long T1 (A), long T2 signal (B), Flair (C) and DWI (D) high signal shadows of the corpus callosum pressure part showed that the child had a high signal shadow;

Continue the original treatment, dexamethasone after 7 days and gradually stop.

Blood sodium was normal
on the 10th day of hospitalization.
On the 14th day of hospitalization, CSF was reviewed, WBC 47 X106/L, lymphocytes 93%, monocytes 7%, protein, sugar, chloride content normal; Discontinue antiviral therapy
.
On the 16th day of hospitalization (20th day after onset), no abnormalities were found on the MRI of the head, and the abnormal signal shadow of the pressure part of the corpus callosum disappeared (Fig.
2).
Recovered and discharged from the hospital
.

Fig.
2 MRI was re-examined on the 20th day of onset, and no abnormalities were found; The original Tl(A), T2(B), Flair(C) and DWI(D) abnormal signal shadows of the corpus callosum pressure disappeared.

DISCHARGE DIAGNOSIS: VIRAL MENINGOENCEPHALITIS, RESLES
.
After 5 months of follow-up after discharge, the child had no abnormalities
.

2 Discussions

Previous reports of RESLES have included "mild encephalitis or encephalopathy with reversible corpus callosum pressure lesions"
, "reversible focal corpus callosum lesions" or "transient corpus callosum pressure lesions".
In 2011, Garcia-Monco et al.
proposed the name
of "reversible corpus callosum pressure lesion syndrome".
So far, only a few adult cases1 have been reported in China, but none of them have used the name and concept of RESLES, and no cases of RESLES in children have been reported
.

The clinical history and prognosis of RESLES are good, MRI is mainly oval long T1, long T2 and DWI high-intensity lesions in the pressure of corpus callosum, and it has also been reported that lesions can affect the entire corpus callosum and even bilateral symmetrical white matter, but disappear and return to normal
in a short period of time.

This case has fever, headache, meningeal irritation, decreased blood sodium, CSF leukocytosis (mainly lymphocytes), elevated protein, normal sugar, chloride and culture, VCA-IgG (+), normal head CT and EEG, consistent with the diagnosis
of viral meningitis.
The children treated with antiviral, sodium supplementation and dexamethasone did not improve, and on the 8th day of onset, cranial MRI showed oval long Tl, long T2, Flair and DWI hyperintensity lesions
in the pressure of the corpus callosum.

After continuing the original treatment, the child's symptoms disappeared
after l d.
Blood sodium returned to normal on the 10th day of onset, the number of CSF cells on the 14th day was significantly reduced, biochemical and normal, and antiviral and sodium supplementation therapy
was stopped.
On the 20th day of onset, there was no abnormality in the MRI of the head, and the abnormal signal lesions of the pressure of the corpus callosum disappeared.
Recovered and discharged from the hospital
.
After 5 months of follow-up after discharge, the child had no abnormalities
.
THE CLINICAL AND RADIOGRAPHIC FINDINGS OF THE CHILD ARE CONSISTENT WITH THE DIAGNOSIS
OF RESLES.

Garcia-Monco et al.
summarized the etiology of 113 patients with RESLES reported from 1966 to 2007, including epilepsy and its related (43.
36%), infection-related (33.
63%), metabolism-related (5.
31%), high-altitude cerebral edema (7.
80%) and other rare causes (10.
62%)
。 Epilepsy and its related causes include seizures, withdrawal from antiepileptic drugs and antiepileptic drugs, most often in patients withdrawing from antiepileptic drugs (89.
80%), but also including patients taking antiepileptic drugs for other reasons; The most frequent antiepileptic drugs are carbamazepine, phenytoin, lamotrigine, as well as valproic acid, oxcarbazepine, topiramate, levetiracetam, etc
.
Among the causes of infection, influenza virus is the most common (18.
42%), and others include rotavirus, adenovirus, mumps virus encephalitis or encephalopathy
.
Metabolic-related causes include hypoglycemia (50%) and hypernatremia (50%)
.
Other rare causes include anorexia nervosa, peroneal muscular atrophy, systemic lupus erythematosus, vitamin B12 deficiency, etc.
, but most of them are reported
in isolated cases.
Unlike adults, the main cause of RESLES in children is central nervous system infection, which is most
commonly reported in Japan.

In this case, the RESLES is caused by
Epstein-Barr viral meningitis.
In this case, the RESLES is caused by
Epstein-Barr viral meningitis.
The pathogenesis of RESLES is not well understood, and due to the many causes of RESLES, its pathogenesis will also be multiple
.
CHILDREN WITH RESLES CAUSED BY VIRAL ENCEPHALITIS ARE OFTEN ACCOMPANIED BY HYPONATREMIA, WHICH CAUSES CHANGES IN OSMOTIC PRESSURE AND CAUSES CEREBRAL EDEMA, SO IT IS THOUGHT THAT RESLES IS RELATED TO CEREBRAL EDEMA
.

In this case, there was no vomiting after the onset of the disease, and the blood sodium decreased at the beginning of the disease, and the occurrence of low blood sodium may be related to
abnormal secretion of antidiuretic hormone caused by infection.
As the condition improved, the blood sodium returned to normal, and the abnormal signal lesions of the corpus callosum pressure on MRI disappeared and returned to normal
.
Suggests that hyponatremia may play an important role
in the pathogenesis of RESLES.
Since most patients with RESLES have a good prognosis, there is a lack of reports of autopsy pathology studies; CRANIAL MRI OBSERVATION STUDIES HAVE SHOWN THAT CEREBRAL EDEMA IN RESLES INCLUDES VASOGENIC AND CYTOTOXIC EDEMA
.

The diagnosis of RESLES needs to be differentiated from neurological demyelinating disease, both of which are associated with viral infections, but treatment and prognosis are different
.
Understanding the clinical and radiographic features of RESLES, as well as observing its dynamics, helps distinguish it from neurological demyelinating disease
.
In short, the causes of RESLES in children are mostly central nervous system viral infections, clinically manifested as symptoms of encephalitis, meningitis or encephalopathy, which may be accompanied by low blood sodium, and imaging features are reversible lesions in the pressure of the corpus callosum, and the prognosis is mostly good
.