Dr Samar Al Muntaser Background There is a paucity of literature - - PowerPoint PPT Presentation

Dr Samar Al Muntaser

Background

• There is a paucity of literature on the epidemiology, clinical characteristics

and investigation tools for the pediatric age group with acquired demyelination syndromes.

• Any disease that causes damage to the myelin sheath that slows or stops

nerve signals is called a demyelinating disease. There are numbers of demyelinating diseases in childhood. Causes are multifactorial and include genetic, post infectious, post-immunization, and autoimmune (the body produces proteins, which damage its own tissue).

• No cure exists for demyelinating diseases; early recognition, supportive care

and early treatment with medications may help minimize and manage

• symptoms. Rehabilitation therapies are of particular importance.

Audit Objectives:

• To determine the early symptoms of demyelinating

syndromes.

• To identify the types of demyelination
• To assess the clinical investigation, MRI features of

childhood demyelinating syndromes.

• To evaluate the treatment modalities.

Methods:

• It is a retrospective study done on all pediatric patients

admitted with demyelinating syndromes from January 2010 until Jan 2019.

• Data was collected from the patients records after

approvals.

• Patients were assessed regarding clinical presentation,

investigation and management plans on regular basis.

Results:

• Total numbers of patients diagnosed as demeylination

syndrome: 13 patients.

• Their age range at presentations : 29 months to 11 6/12

years.

• 8 Patients were female and 5 males
• All patients had normal birth history , normal development

and family history.

• Triggering factors: 8 cases presented with history of fever
• r viral infection

Clinical presentations :

NECK PAIN / STIFFENESS DROWSY HEADACHE MOTOR TICS IRRITABLE NIL 4 (30.7%) 3 (23%) 2 (15%) 1 (7.7%) 1 (7.7%) 1 (7.7%)

CNS examination:

• Cranial nerves:

1 patient with squint and diplopia (7.6%) 1 patient with left facial weakness (7.6%)

• Power:

6 patients with weakness (46%)

• Tone:

1 was hypertonic (7.6%), 1was hypotonic (7.6%) and 84.6% were normal

• Reflexes:

9 patients had brisk reflexes ( 69%), 7.6% hyporeflexia and 7.6% absent reflex

• Gait
• 7 patients had unsteady gait (53.8)

Investigations :

• CSF: 1 patient with positive HSV1

3 with high protein

• OCB/CSF:

3 were positive 3 were not done 7 were negative

• AQ4:

5 negative 8 not done

Investiagtions :

• EEG:

4 patients had focal slowing (30.7%) 1 epileptiform discharges (7.6%) 1 not done ( 7.6%) 7 were normal (53.8%)

• MRI:

7 patients with signs of ADEMS (53.8%) 6 patients with signs of transverse myelitis (46%)

Diagnosis:

Management

Conclusions

• Though demyelinating syndromes is rare in children , yet

it seems to be under diagnosed due to paucity of investigations tools.

• Re-audit of new patients prospectively

with possible demyelination to implement

• ther

investigations tools not used previously, to standardize treatment care.