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With refere..
Answer: A = Zero
Explanation/Comment: It is heartening to note that there seems to be a trend towards better knowledge,
attitudes and practices about epilepsy in recent surveys.
Ref:
M. Shaju et al, Knowledge, attitude and practice of parents regarding pediatric antiepileptic
drug therapy, International Journal of Epilepsy, 1(2014)57–63.
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With..
Answer: B = Alcohol Abuse
Explanation: Alcohol use could be infrequent in the muslim population of Nigeria.
In other developing countries too, the remaining alternatives cause are more fragment
precipitating factor than alcohol abuse.
Ref.: LF Owolabi et al, Status epilepticus in adults; A study from Nigeria, International
Journal of Epilepsy 1(2014) 69–74.
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While comparing..
Answer: D = Abnormal neuro electrical activity
Comment: The definition of neonatal seizures is purely clinical, hence entirely arbitrary
and resulting in over as well as under diagnosis.
Source: KP Vinayan, SL Moshe, Neonatal seizures and epilepsy, International Journal of Epilepsy
1(2014) 75–83.
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Which of the following…
Answer: A = Multifocal or fragmentary myoclonus
Comment: Multifocal or ‘fragmentary’ myoclonus is characterized by brief asynchronous twitching
of different muscle groups. Of the three types, fragmentary myoclonus is the type
least commonly associated with EEG changes.
Source: KP Vinayan, SL Moshe, Neonatal seizures and epilepsy, International Journal of Epilepsy
1(2014) 75–83.
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Many factors contribute to..
Answer: b = Low concentration of extracellular potassium in immature brain
Explanation: Developmental imbalance between the maturation of excitatory and inhibitory circuits
is supposed to be the most important factor for the increased seizures burden in the
newborn. Immature brain has HIGH concentration of extracellular potassium, which may
contribute to the increased excitability. The newborn brain is also vulnerable to
a variety of insults like hypoxia and hypoglycemia, further increasing the chance
of seizures.
Source: KP Vinayan, SL Moshe, Neonatal seizures and epilepsy, International Journal of Epilepsy
1 (2014) 75–83.
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Motor automatisms, …
Answer: D = Sustained eye opening and fixation
Explanation: Motor automatisms, previously called subtle seizures may usually occur in encephalopathic
neonates. These are very difficult to characterize without simultaneous EEG. In preterm
infants, the most common manifestation is sustained eye opening with unresponsiveness
and eye fixation. In full term infants, horizontal sustained deviation of the eyes
is usually seen.
Slow roving eye movements and orofacial movements may be physiological and are very
difficult to differentiate from subtle seizures. Jittery or tremulous movements are
usually seen in metabolic and electrolyte anomalies and drug withdrawal. They are
stimulus sensitive and may be modified by positioning. Hyperekplexia, a genetic disorder
due to a mutation in glycine receptor gene, is characterized by stimulus sensitive
myoclonus, muscle rigidity, episodes of tonic spasms and rare apnea.
Source: KP Vinayan, SL Moshe, Neonatal seizures and epilepsy, International Journal of Epilepsy
1(2014) 75–83.
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Bening Familial Neonatal Convulsions…
Answer: A = Potassium Channel
Explanation: Benign neonatal epilepsies are characterized by transient seizures and good neuro-developmental
outcome. There are two types; benign familial neonatal seizures and benign non familial
neonatal seizures. Benign familial neonatal seizures otherwise called benign familial
convulsions (BFNC) is an autosomal dominant potassium channelopathy affecting the
KCNQ2 and KCNQ3 genes.
Source: KP Vinayan, SL Moshe, Neonatal seizures and epilepsy, International Journal of Epilepsy
1(2014) 75–83.
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Early Infantile Epileptic Encephalopathy (…
Answer: D = Inborn errors of metabolism
Explanation: Early Infantile Epileptic encephalopathy (EIEE, Ohtahara syndrome) and the early
myoclonic epileptic encephalopathy (EMEE, Aicardi syndrome) are the two named epileptic
encephalopathies with onset in the neonatal period. Both the syndromes are associated
with resistant epilepsies and poor neuro-developmental outcome along with suppression
burst pattern in the EEG. Ohtahara syndrome is usually seen with severe structural malformations of the brain and the main seizures type is tonic spasms from the beginning. On the other hand, EMEE is usually associated with inborn errors of metabolism. The interburst interval is longer in EIEE compared to EMEE. Some times, the burst
suppression pattern is in EMEE may not be appreciated at disease onset, and follow
up EEGs may be necessary to make the diagnosis. Seizures are typically refractory
to currently available anticonvulsant medications. Outcome is uniformly poor for both
the syndromes with a high rate of mortality in early infancy. Surviving children may
develop hypsarrhythmia at around 3–6 months with classical features of west syndrome.
Source: KP Vinayan, SL Moshe, Neonatal seizures and epilepsy, International Journal of Epilepsy
1(2014) 75–83.
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All True statement about Levetiracetam, …
Answer: C = Mostly metabolized in liver, with minimal renal elimination
Explanation: Levetiracetam (LEV) is a novel anti-epileptic drug having a broad spectrum anti-seizures
activity in both generalized as well as focal onset seizures. It is a relatively well-tolerated
anti-epileptic drug (AED) in both adults and children. It is a new AED with renal
elimination and no hepatic metabolism.
Source: R. Mahale et al, Possible Levetiracetam induced encephalopathy presenting as electrical
status epilepticus: An unknown occurrence, International Journal of Epilepsy I(2014)
84–89.
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Two types of GM2 Gangliosidosis (Tay-Sachs and…
Answer: A = Organomegaly
Explanation: Differentiation of the two subtypes (Tay-Sachs and Sandhoff’s) of GM2 gangliosidosis
is biochemically not possible if the facility for serum total hexosaminidase levels
testing are not available.
In GM2 gangliosidosis the radiological changes are described in three phases. Initially
there is enlargement with abnormal signal changes of basal ganglia (particularly caudate)
followed by features of hypomyelination with diffuse cerebral atrophy in the terminal
stages.
Clinically patients with classic GM2 gangliosidosis present with infantile onset psychomotor
regression, hyperacusis, macular cherry red spot, central hypotonia and blindness.
As the disease progresses megalencephaly and generalized seizures are seen. Sandhoff’s
disease in addition exhibits hepatosplenomegaly and cardiomyopathy.
Source: R. Dubey et al, A child with global neuroregression with refractory epilepsy, International
Journal of Epilepsy 1(2014) 92–93.
