Dr Rithwik Sunil, Dr. Vinayan, Dr. Sajith Kesavan,Dr C Jayakumar
Seven year old child, 1st of NCM, developmentally normal and immunized presented with bilateral lower limb weakness followed upper limb weakness and left sided facial weakness
To begin with he had upper respiratory symptoms but progressed to breathlessness .
On 4th day of illness child had features of7th ,9th and 10th nerve involvement . Parents also noticed loss of tone, volume and clarity of speech. Subsequently he developed hypotonia and reduced power of both upper and lower limbs. DTR were absent in the lower limb and plantar was down going. Single breath count was 14
Child was ventilated electively on7th day and as the power improved he was extubated on day 11th
Differentials
Guillan Barre syndrome
Tick paralysis
SLE
Acute disseminated encephalomyelitis
Toxic peripheral neuropathy
Acute cerebellar ataxia syndrome
Diphtheria
Lymes disease
Thiamine deficiency
Acute viral fever with myalgia
LABS
CBC/CRP/RUE/RFT/LFT normal
Mycoplasma IgM positive
Other viral panel negative
MRI brain shows no focal enhancing lesions or meningeal enhancement
Mycoplasma IgM positive
Other viral panel negative
MRI brain shows no focal enhancing lesions or meningeal enhancement
CSF cytology : lymphocytic pleocytosis
Lymphocytes(11-15/hpf)
Protein 1049 mg%
Glucose74.6mg%
No atypical cells seen
She was shifted out to HDU in hemodynamically stable condition with normal neck flexors and upper limb power and improving bulbar and respiratory muscle weakness
Plan was to follow the child and continue therapies and moniter her clinical course
Child underwent elective intubation in view of the risk of respiratory failure due to the ascending paralysis
Single breath count was 14
Eventually weaned off respiratory support day 11
Ganglioside antibody evaluation panel in serum with IgM and IgG were sent
Serum sample sent tested positive for GD1b and GQ1b
NCV: showed evidence of predominantly demyelinating type of sensory motor neuropathy involving lower and upper limbs.
Child was then started on IVIg 400mg/kg for 5 days
Since then Child and undergoing physio therapy
The Guillain-Barré syndrome (GBS) is an acute immune-mediated polyneuropathy considered as the most common causes of acute flaccid paralysis in healthy children . GBS is more frequent in children aged 1-5 yr. The syndrome is more prevalent among males than females. GBS is thought to be an autoimmune disorder that results from T and B cell activated immune response to some preceding infectious agents such as Campylobacter jejuni, Cytomegalovirus, Epstein-Barr virus, Mycoplasma pneumoniae and HIV. Due to molecular mimicry of such infections, they could have cross-reaction with peripheral nerve components including the myelin or the axon, which consequently forms different types of GBS. Some triggering factors such as immunization, surgery, trauma, and bone-marrow transplantation could have role in the pathogenesis of GBS .
Even though mycoplasma and campylobacter are reasons for GBS treatment for the same with antibiotics will not prevent GBS
Though results of different studies from various geographical regions have reported great variability regarding the epidemiology and clinical features of GBS, the most frequent clinical presentations of GBS in children are pain, progressive muscle weakness, and reduced deep tendon reflexes. In younger children aged less than 4 yr, the most common features are pain in the legs and refusal to walk .
TPE(Therapeutic plasma exchange) can be performed safely in pediatric patients of GBS and has been shown to be effective as first line or adjunctive therapy in selected diseases. It shortens the course of hospitalization and reduces the mortality and incidence of permanent paralysis. It is a safe procedure when volume shifts, calcium supplementation, venous access; anticoagulation and psychosocial aspects are taken care of. Occasionally, minor complications such as hypotension and shivering occur.
Take home message
It is important to understand and be able to obtain an early diagnosis in GBS as the condition most often eventually affects the respiratory muscles and could lead to respiratory failure. All patients with Guillain-Barré syndrome (GBS) require close monitoring of motor, autonomic (ie, blood pressure, heart rate, and sphincter function), and respiratory function during the acute phase of the illness