First clinical attack of inflammatory or demyelinating disease in the CNS. Alteration in consciousness ranging from somnolence or coma

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2 ADEM Clinical features First clinical attack of inflammatory or demyelinating disease in the CNS Acute or subacute onset Affects multifocal areas of the CNS Polysymptomatic presentation Must include encephalopathy: Acute behavioral change such as confusion or irritability and/or Alteration in consciousness ranging from somnolence or coma Attack should be followed by improvement on clinical and/or neuroradiologic (MRI) measures Sequelae may include residual deficits No other etiologies can explain the event ADEM relapses (with new or fluctuating symptoms, signs or MRI findings) occurring within three months of the inciting ADEM episode are considered part of the same acute event. In addition, ADEM relapses that occur during a steroid taper or within four weeks of completing a steroid taper are considered part of the initial inciting ADEM episode. Lesion Characteristics on MRI FLAIR and T2 weighted images Large (>1 to 2 cm in size) multifocal, hyperintense, bilateral, asymmetric lesions in the supratentorial or infratentorial white matter. Rarely, brain MRI shows a single large (1 to 2 cm) lesion predominantly affecting white matter. Gray matter, especially basal ganglia and thalamus, may be involved Spinal cord MRI may show confluent intramedullary lesion(s) with variable enhancement, in addition to the abnormalities on brain MRI No radiologic evidence of previous destructive white matter changes

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6 RISK FACTOR FOR RECURRENCE

7 CRITERIA FOR THE CLASSIFICATION OF JUVENILE RHEUMATOID ARTHRITIS Age at onset: <16 yr Arthritis (swelling or effusion, or the presence of 2 or more of the following signs: limitation of range of motion, tenderness or pain on motion, increased heat) in 1 joints Duration of disease: 6 wk Onset type defined by type of articular involvement in the 1st 6 mo after onset: Polyarthritis: 5 inflamed joints Oligoarthritis: 4 inflamed joints Systemic disease: arthritis with rash and a characteristic quotidian fever Exclusion of other forms of juvenile arthritis

8 Modified from Cassidy JT, Levison JE, Bass JC, et al: A study of classification criteria for a diagnosis of juvenile rheumatoid arthritis, Arthritis Rheum 29; , MACROCEPHALY

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10 HYPOGLYCEMIA In a newborn a value less than 40mgm% is hypoglycemia In a child a value less than 50mg% In a malnoutished (sam) the cut off is 54 mg%

11 MICROPENIS

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13 STAPHYOCOCCAL PNEUMONIA

14 RHEUMATIC FEVER

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21 STATUS EPILEPTICUS Status epilepticus is a medical emergency that should be anticipated in any patient who presents with an acute seizure. It is defined as continuous seizure activity or recurrent seizure activity without regaining of consciousness lasting for >30 min. Some have advocated 5 min (rather than 30) as the time limit, but others have suggested using the term impending status epilepticus for seizures between 5 and 30 min. The measures used to treat status epilepticus need to be started in any patient with acute seizures that do not stop within a few minutes. The most common type is convulsive status epilepticus (generalized tonic, clonic, or tonic-clonic), but other types do occur, including nonconvulsive status (complex partial, absence), myoclonic status, epilepsia partialis continua, and neonatal status epilepticus. About 30% of patients presenting with status epilepticus are having their first seizure, and approximately 40% of these later develop epilepsy. Febrile status epilepticus is the most common type of status epilepticus in children Nonconvulsive status epilepticus manifests as a confusional state, dementia, hyperactivity with behavioral problems, fluctuating impairment of consciousness with at times unsteady sitting or walking (absence status), fluctuating mental status, confusional state, hallucinations, paranoia, aggressiveness catatonia, and psychotic symptoms. Epilepsia partialis continua has been defined previously and can be caused by tumor, vascular etiologies, mitochondrial disease (MELAS), and Rasmussen encephalitis. Refractory status epilepticus is status epilepticus that has failed to respond to therapy, usually with at least 2 (although some have specified 3) medications. Whether there should be a minimum duration has not been agreed upon, as authors have variably cited 30-min, 60-min, or 2-hr durations. New-onset refractory status epilepticus (NORSE) has been identified as a distinct entity that can be caused by almost any of the causes of status epilepticus in a patient without prior

22 epilepsy. It also is often of unknown etiology, presumed to be encephalitic or postencephalitic, can last several weeks or longer, and often has a poor prognosis.

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