Morning Report

Danae Goerl, PGY-3

 Chief

complaint: 9yo previously healthy girl with abnormal movements History of Present Illness:

Symptoms started 9 days ago Started to have abnormal movements

When eating, her fork would shake while guiding to her mouth At school, having a difficult time reading out loud in class Writing has become worse She would randomly wave arms, or kick out her legs Difficulty getting out of bed and walking stairs

HPI continued
Evaluated

at Urgent Care 5 days prior, thought to have behavior issues

ROS:

No fever, no joint pain, no joint swelling, no rash, no headache, no vomiting, +sore throat one month prior

 Past

Medical History: No chronic illness, no previous hospitalizations PSHx: none No daily meds NKDA IMM: UTD

 Social

History: lives at home with parents. In 3rd grade, doing well until recently. No pets History: Father recently diagnosed with seizure disorder. No history of autoimmune disease

Family

PHYSICAL EXAM
Vitals: HR 102, RR 20 T 37.5 BP 104/60 Gen: alert, cooperative with exam HEENT: conjunctiva clear, PERRL, TMs normal, normal oropharynx, no pharyngeal erythema or plaques NECK: no LAD RESP: CTAB, non labored breathing CV: Normal S1, S2. Grade 1/6 high pitched murmur at apex without radiation. No rubs, or gallop. Normal pulses ABD: soft, NT, ND. No hepatosplenomegaly EXT: 3rd, 4th MCP on right hand with limited ROM, and pain with motion. Otherwise joints normal. NEURO: Alert, dysarthric speech, Intermittently shrugs shoulders. Normal tone. Normal sensation. Dysmetria with finger nose finger. Difficulty sustaining grasp. Normal sensation. Normal gait DERM: no rash

Assessement:
9 yo girl with one week of abnormal movements

Differential Diagnosis

Neuro Tic disorder Stroke Intracranial malignancy Tourette syndrome Familial benign chorea Huntington chorea Ataxia teleangictasia Niemann-Pick Lesch Nyhan Rheum SLE cerebritis Syndenhams chorea ID Viral Encephalitis HIV Cardiac Cardiac surgery Post pump chorea

Endo Thyrotoxicosis GI Wilsons disease Psych ADHD with choreoform movements Tardive dyskinesia Metabolic Hypo/hypernatremia Hypoglycemia Hypocalcemia Onc Perineoplastic syndromes Toxins Manganese Methanol Carbon Monoxide

Laboratory: CMP: Normal CBC: WBC: 5.1, Hct 49, Plts 236 diff wnl ESR: 7 CRP: <0.5 ASO: 342 (0-199 IU/mL) DNAse B Ab: 354 (0-170 U/mL)

IMAGING: Echo: 1. Mild to moderate mitral valve regurgitation 2. Normal RV, LV size and function 3. No pericardial effusion
ECG: normal sinus rhythm (normal PR interval)

Diagnosis
Rheumatic

Fever with Sydenhams

Chorea

The presence of chorea alone is sufficient to diagnose ARF

Guidelines for the diagnosis of rheumatic fever. Jones Criteria, 1992 update. Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association. JAMA. 1992;268(15):2069.

Sydenham Chorea
Most

common cause of acquired chorea in childhood 10-40% of children with ARF have chorea Carditis more commonly associated with Sydenham Chorea than arthritis
Epidemiology

Most common in ages 5-15 2:1 female predominance

1. Zomorrodi A, Wald ER. Sydenham's chorea in western Pennsylvania. Pediatrics. 2006. 2. Veasy LG, Tani LY, Hill HR. Persistence of acute rheumatic fever in the intermountain area of the United States. J Pediatr. 1994;

Presentation Can be weeks to months after strep infection Insidious onset, clumsiness, behavior changes Progressive chorea Milk maids grasp Darting tongue hung-up tendon jerk reflexes (ie, brisk reflex followed by a slowed return to the neutral position).

Weeks to months course, ultimate resolution

Video

Diagnostic Testing in Chorea

Most ASO, Anti DNase B +, but 10-25% can be

ASO peaks at 4-5 weeks, DNase B elevated for months

Throat culture Antistreptolysin O titer (ASO) AntiDNase B titer Electrocardiography Echocardiography Thyroid function tests Complete blood count Antinuclear antibody Erythrocyte sedimentation rate Magnetic resonance imaging of brain Serum ceruloplasmin concentration Antiphospholipid/anticardiolipin antibodies Urine drug screen Urine human chorionic gonadotropin concentration

Treatment
Secondary

prevention

Penicillin AAP guidelines Treatment until age 21

Symptomatic

treatment of chorea

Neurology referral Carbamazepine vs valproic acid 2-4 months

Immune

modulation

4 weeks of prednisone 2mg/kg/day, then taper

Paz JA, Silva CA, Marques-Dias MJ Randomized double-blind study with prednisone in Sydenham's chorea. Pediatr Neurol. 2006;34(4):264.

Treatment Continued