|Year : 1965 | Volume
| Issue : 3 | Page : 119-120
Schilder's disease- encephalitis periaxialis diffusa
Military Hospital, Bangalore-7, India
|Date of Web Publication||22-Feb-2008|
N C Singhal
Military Hospital, Bangalore-7
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Singhal N C. Schilder's disease- encephalitis periaxialis diffusa. Indian J Ophthalmol 1965;13:119-20
Schilder's disease is a rare demyelinating disorder affecting chiefly the subcortical white matter of cerebral hemisphere. It generally affects infants and children and is characterised by progressive spastic paralysis, cortical blindness and death.
| Case Report|| |
Pushpa a female child aged six years attended the Eye Department of Wellington hospital on 8th Jan. 1959 with a complaint of dimness of vision in the right eye for about two months.
On examination there was central corneal opacity in the right eye and a right divergent squint of about 10 to 15 degree. The child could not count fingers with that eye, whereas she could do that with the left eye. The right eye pupil was dilated and was reacting sluggishly to light, probably because of previous use of a mydriatic at another hospital. The fundus was normal. The left pupil and fundus were normal. No satisfactory decision could be arrived at as to the diagnosis.
On 28th Jan. 1959 the child was brought again to the department with the complaint that now she could not see with the left eye also. This was detected by the parents when the child while walking in the house struck against objects lying on the floor and fell down. Another complaint was that the child wept for the last one week whenever her head was combed and had been sitting idle. History of mild fever could be elicited with difficulty from the parents.
On examination both pupils were normal in size, shape and reaction to light both in speed and range. Fundii were normal. The child did not reply when asked to count fingers and was unable to co-operate for testing ocular movements which however appeared to be normal. She appeared to be listless and was not interested to catch even toys, unless put in her hands. There was no menace reflex.
The central nervous system and other systems on examination were found normal. Physically and mentally the patient was normal till the recent illness. There was no birth or developmental defect.
Examination of the blood revealed a low R.B.C. count (2.6 million) with 50% hoemoglobin. The white blood count, total and differential was normal. Blood sedimentation rate (Wester- green) was 60 mm in one hour. The cerebro-spinal fluid was under normal pressure, and nothing abnormal was detected in its examination. Stool and urine examinations, X-Ray of the skull and encephalography showed nothing abnormal.
The child was put on prednisolone by mouth on an empirical basis and was given Inferon for her anaemia. There was no change in the condition even after 15 days of treatment.
In view of the normal pupils, normal fundi, total loss of vision, age and investigation results within normal limits, a diagnosis of Schilder's disease was made. A course of Histamine diphosphate injections one mgm intramuscularly bi-weekly was advised, increasing the dose by one mgm every week for one month.
The child was again seen on the 6th March 1959. There was no change in the eye condition. On the contrary, nerve deafness was suspected but could not be confirmed because of the child's inability to respond. It had become more dull and apathetic now.
A month later the child suddenly fell to the floor and was put to bed. Since then she could not stand but could sit. On examination, there was spastic paralysis of the legs. The upper extremities were similarly affected but to a lesser extent. Her appetite became poor. On the 7th day of confinement to bed parents reported that there was incontinance of urine and stools and she was daily getting worse. The child died on 25th April.
No autopsy was done.
| Discussion|| |
Schilder described the disease in 1912 and since then the diagnosis has been made in many instances only at autopsf, which emphasizes the difficulty in diagnosis. Brain and Strauss state that there is no other disease affecting young adults and children in which the following triad is present.
- Progressive loss of vision.
- Progressive spastic paralysis.
- Progressive mental deterioration.
These three features may be present simultaneously, when the condition is rapidly fatal, or independently, the other features developing subsequently. Because of extreme rarity of Schilder's disease, its diagnosis on symptomatology of progressive spastic paralysis or progressive mental deterioration alone will seldom be thought of because there are other conditions which are initiated with similar symptomatology. The logical conclusion of progressive loss of vision without pupillary disturbances is that the lesion is cortical and the only possibility of such a lesion is Schilder's disease. Other possible lesions are vascular and tumours which will have their own features for diagnosis. Investigations like X-ray skull, lumber puncture, encephalography, would no doubt be done but all these are likely to be normal. Diagnosis can only be confirmed by autopsy.
The common modes of onset of the disease may be as follows:
- Progressive mental deterioration in the form of irritability, peevishness, unprovoked laughing.
- Disturbances of motility such as hemiplegia unilateral or bilateral. monoplegia and cortical paraplegia.
- Cortical blindness may be the first symptom.
The blindness is due to extensive symmetrical lesions in the optic radiations of both cerebral hemispheres. Sensory symptoms are difficult to elicit because of mental changes but any variety of sensory lesions may be found.
Headaches occur frequently. Rarely optic nerves may be attacked by the process, but when they are involved, choked discs add to the diagnostic problem. These signs and symptoms lead to frequent diagnosis of cerebral tumours and unsuccessful exploratory operations performed in these patients.
The present case began with cortical blindness and developed later into cortical deafness and disturbances of motility. Death came earlier than mental symptoms.
| Summary|| |
A case of Schilder's disease is described. A brief review of the literature is given.
| References|| |
Grinker, Bucy and Sahs (1959), "NEUROLOGY" p. 967 Blackwell scientific publication, Oxford.
Walsh F.B. (1957) "Clinical Neuro-ophthalmology" 2nd edition p. 662, The Williams and Wilkins Co. Baltimore.