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USMLE Pediatrics > 3 > Flashcards

3 Flashcards

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USMLE® Step 1 flashcards
1
Q

2–year–old boy developed anemia after treatment with trimethoprim–sulfamethoxazole 3 weeks earlier. Hb and Hematocrit 7.2 g/dL and 24%. What is the diagnosis?

A

Glucose–6–phosphate dehydrogenase (G6PD) deficiency.

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2
Q

What is glucose–6–phosphate dehydrogenase deficiency?

A

Episodic hemolytic anemia. X–linked. Mediterranean, Middle Eastern, African, Asians. 24 hours after ingestion of oxidant (aspirin, sulfa drugs, antimalarials, fava), infection, illness. Rapid drop Hb, hemoglobinuria, jaundice. Reticulocytosis.

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3
Q

How is glucose–6– phosphate dehydrogenase deficiency diagnosed?

A

Direct measurement of G6PD activity.

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4
Q

What is the treatment for glucose–6–phosphate dehydrogenase deficiency?

A

Avoid oxidants, broad beans (ie, fava beans), primaquine, chloroquine, nitrofurantoin, ciprofloxacin, Pyridium, sulfonamides.

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5
Q

8–month–old, African–American infant with painful swollen hands and swollen feet. What is the diagnosis?

A

Sickle cell anemia.

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6
Q

What is the cause of sickle cell anemia?

A

Single base pair change (thymine for adenine) at the sixth codon of the beta gene (valine instead of glutamic acid).

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7
Q

What is the clinical presentation of sickle cell anemia?

A

Hemolytic anemia develops over first 2–4 months with the replacement of hemoglobin F. Hand–foot syndrome (dactylitis), which is a symmetric, painful swelling of hands, feet (ischemic bones).

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8
Q

What are acute sickle painful crises?

A

Characterized by pain in the extremities in younger. With increasing age, there is pain in the head, chest, back, abdomen.

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9
Q

What factors may precipitate a sickle painful crisis?

A

Precipitated by illness, fever, hypoxia, acidosis, or no precipitating factor can be identified.

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10
Q

What are the signs of vasoocclusive crises in sickle cell disease?

A

Skin ulcers, retinopathy, avascular necrosis of hip, infarction of bone and marrow (Salmonella osteomyelitis); splenic autoinfarction; acute chest syndrome; stroke (6–9 y); priapism in adolescence.

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11
Q

What is acute splenic sequestration?

A

Altered splenic function results in increased susceptibility to infection, especially with encapsulated bacteria (S. pneumococcus, H. influenzae, N. meningitidis). Peak 6 months to 3 years of age; can rapidly lead to death.

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12
Q

What is a sickle cell a plastic crisis?

A

Aplastic crisis occurs after infection with parvovirus B19; absence of reticulocytes during acute anemia.

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13
Q

How does sickle cell disease affect the kidneys?

A

Decreased renal function (proteinuria is the first sign); urinary tract infections.

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14
Q

What are the laboratory abnormalities in sickle cell anemia?

A

Increased reticulocytes, moderate anemia, normal MCV, nucleated RBCs. Blood smear shows target cells, poikilocytes, hypochromasia, sickle RBCs, nucleated RBCs, Howell–Jolly bodies (lack of splenic function). Bone marrow is hyperplastic.

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15
Q

How is sickle cell anemia diagnosed?

A

Newborn screening for hemoglobin S. Confirm diagnosis with hemoglobin electrophoresis. Prenatal diagnosis for parents with trait.

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16
Q

What is the treatment for sickle cell anemia?

A

Immunize with routine vaccines plus pneumococcal vaccine. Start penicillin prophylaxis at 2 months. Folate. Pain control; transfuse as needed. Oral hydroxyurea for patients with frequent painful crises. Bone–marrow transplant.

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17
Q

What is beta thalassemia major?

A

Excess alpha globin chains causes alpha tetramers to form; increase hemoglobin F. Presents in 2nd mth with anemia, hypersplenism, cardiac decompensation. Expansion of skull: extramedullary hematopoiesis; HSM.

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18
Q

What are the laboratory abnormalities in beta thalassemia major?

A

Infants with HbF only, which is detected by electrophoresis. Severe anemia, low reticulocytes, increased nucleated RBCs, hyperbilirubinemia, microcytosis. Increased ferritin and transferritin saturation. Marrow hyperplasia.

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19
Q

What is the treatment for beta thalassemia major?

A

Transfusions, deferoxamine, splenectomy. Bone–marrow transplant is curative.

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20
Q

What type of bleeding occurs in von Willebrand disease (vWD) or platelet dysfunction?

A

Mucous membrane bleeding, petechiae, small ecchymoses.

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21
Q

What type of bleeding occurs in clotting factor deficiency?

A

Deep bleeding with extensive ecchymoses and hematoma.

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22
Q

What is the initial evaluation of bleeding disorders?

A

Obtain platelets, bleeding time, PT, PTT. If initial tests are normal, check von Willebrand factor and thrombin time. If abnormal, obtain a clotting factor evaluation.

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23
Q

What is the purpose of testing bleeding time?

A

Tests platelet function and interaction of platelets with vessel walls; detects qualitative platelet defects or lack of von Willebrand factor.

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24
Q

What is the most common acquired cause of bleeding disorders in children?

A

Thrombocytopenia

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25
Q

What is the partial thromboplastin time?

A

Test of the intrinsic pathway. Assess from initiation of clotting at level of factor XII through the final clot (prolonged with factor VIII, IX, XI, XII deficiency).

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26
Q

What is the prothrombin time?

A

Evaluates the extrinsic pathway after activation of clotting by thromboplastin in the presence of calcium; prolonged by deficiency of factors VII, XIII, or anticoagulants; values are standardized with the International Normalized Ratio.

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27
Q

What is the thrombin time?

A

Measures the final step of coagulation, which is fibrinogen conversion to fibrin; a prolonged test indicates decreased fibrin or abnormal fibrin or substances that interfere with fibrin polymerization (heparin, fibrin split products).

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28
Q

What is the characteristic of von Willebrand disease bleeding?

A

von Willebrand disease causes only minor bleeds. Deep bleeds are usually caused by hemophilia.

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29
Q

1–year–old with excessive bruising, epistaxis. Family history of bleeding. What is the diagnosis?

A

Hemophilia

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30
Q

What percentage of hemophiliacs have hemophilia A?

A

85% have hemophilia A; and 15% have hemophilia B; no racial or ethnic predisposition.

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31
Q

What is hemophilia?

A

X–linked disorder of delayed clot formation. Easy bruising with onset of crawling. Hemarthroses first in ankles; knees and elbows. Large–volume blood loss into iliopsoas muscle causes inability to extend hip, vague groin pain, shock.

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32
Q

What are the laboratory findings in hemophilia?

A

2 times normal increase in PTT (other coagulation tests are normal). Normal serum causes correction of PTT to normal. Specific assay confirms diagnosis. Normal platelets, PT, bleeding time, and vW factor.

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33
Q

What is the treatment for hemophilia?

A

Replace factor. Prophylaxis for children with a history of severe bleeding prevents chronic joint disease. For mild bleed, endogenous factor can be released with desmopressin (intranasal). Avoid aspirin.

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34
Q

What is von Willebrand disease?

A

Most common hereditary bleeding disorder; autosomal dominant, but more females are affected. vWF normally adheres to subendothelial matrix, and platelets then adhere to the matrix and become activated.

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35
Q

What is the clinical presentation of von Willebrand disease?

A

Mucocutaneous bleeding, excessive bruising, epistaxis, menorrhagia, postoperative bleeding.

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36
Q

What are the laboratory findings in von Willebrand disease?

A

Increased bleeding time and PTT. Quantitative assay for vWFAg, vWF activity (ristocetin cofactor activity), plasma factor VIII, determination of vWF structure. Normal platelet count.

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37
Q

What is the treatment for von Willebrand?

A

Increase the level of vWF and factor VIII. DDAVP induces release of vWF. Plasma–derived vWF is given.

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38
Q

3–year–old child with petechiae, purpura, and excessive bleeding after falling. What is the diagnosis?

A

Immune thrombocytopenic purpura.

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39
Q

What is immune thrombocytopenic purpura?

A

Autoantibodies against platelet surface. 1–4 weeks after a nonspecific viral infection.

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40
Q

What is the presentation of immune thrombocytopenic purpura?

A

Most patients are 1–4 years of age. Sudden onset of petechiae, purpura, mucousal bleeding. Resolves within 6 months. No HSM or lymphadenopathy.

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41
Q

What are the laboratory findings in immune thrombocytopenic purpura?

A

Platelets

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42
Q

What is the treatment for immune thrombocytopenic purpura?

A

Platelet transfusion contraindicated unless there is life–threatening bleeding (platelet antibodies bind to transfused platelets). IV immunoglobulin for 1–2 days. If inadequate response, then prednisone. Splenectomy for severe disease.

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43
Q

6–year–old with a limp, low–grade fever, URI symptoms, hepatosplenomegaly, and petechiae. What is the diagnosis?

A

Acute lymphoblastic leukemia

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44
Q

What is the most common childhood leukemia?

A

Lymphoblastic leukemia. 77% of all childhood leukemias.

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45
Q

What is the presentation of acute lymphoblastic leukemia?

A

Onset brief and nonspecific. Bone and joint pain, especially lower extremities. Bone marrow failure causes pallor, bruising, epistaxis, petechiae, purpura, mucous membrane bleeding, lymphadenopathy, HSM, joint swelling.

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46
Q

How is acute lymphoblastic leukemia diagnosed?

A

Anemia, thrombocytopenia. WBC is usually less than 10,000/mm3 (atypical lymphocytes). Bone marrow aspirate shows lymphoblasts.

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47
Q

What is the treatment for acute lymphoblastic leukemia?

A

Remission induction (98% remission) with vincristine, daunorubicin and prednisone. Central nervous system treatment with methotrexate. Intrathecal chemotherapy.

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48
Q

What are the complications of acute lymphoblastic leukemia?

A

Relapse occurs in 20%. Increased intracranial pressure or isolated cranial nerve palsies. Testicular relapse in 1–2% of boys. Pneumocystis pneumonia. Opportunistic infections.

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49
Q

What is tumor–lysis syndrome?

A

Initial chemotherapy causes cell lysis, hyperuricemia, hyperkalemia, hypophosphatemia. Low phosphate causes hypocalcemia (tetany, arrhythmias, renal calcinosis).

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50
Q

What is the treatment of tumor–lysis syndrome?

A

Hydration, alkalinization of urine, prevent uric acid formation.

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51
Q

14–year–old male with weight loss, fever, and night sweats. Nontender cervical lymph node 4–5 cm. What is the diagnosis?

A

Hodgkin lymphoma

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52
Q

What is the Hodgkin lymphoma?

A

Ebstein–Barr virus and immunodeficiencies predispose to Hodgkin lymphoma. Diagnostic hallmark is the Reed–Sternberg cell (large cell with multilobulated nuclei). Occurs in 15– to 19–year–olds.

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53
Q

What is the clinical presentation of Hodgkin lymphoma?

A

Painless, firm cervical or supraclavicular nodes. Anterior mediastinal mass. Night sweats, fever, weight loss, lethargy, anorexia, pruritus.

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54
Q

How is Hodgkin lymphoma diagnosed?

A

Excisional biopsy of the node. Staging from I to IV (single node to diffuse disease).

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55
Q

What is the treatment for Hodgkin lymphoma?

A

Chemotherapy with ABVD: Adriamycin, Bleomycin, Vinblastine, and Dacarbazine. Radiation.

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56
Q

What is the prognosis for Hodgkin lymphoma?

A

Overall cure rate of 90% with early stages and >70% with more advanced disease.

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57
Q

What is non–Hodgkin lymphoma?

A

Malignant proliferation of lymphocytes of T–cell, B–cell, or intermediate–cell origin. Epstein–Barr virus causes Burkitt lymphoma. Predisposition by congenital or acquired immunodeficiencies.

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58
Q

What is the presentation of non–Hodgkin lymphoma?

A

Anterior mediastinal mass which may cause wheezing and pneumonia. Abdominal pain, abdominal mass. Spread via blood.

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59
Q

How is non–Hodgkin lymphoma diagnosed?

A

Biopsy. Chest x–ray, CBC, ESR, CT scan of chest and abdomen; bone marrow biopsy. Staging I to IV (localized to disseminated; CNS or bone marrow).

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60
Q

What is the treatment for non–Hodgkin lymphoma?

A

Surgical excision of abdominal tumors, chemotherapy, and monoclonal antiCD20. Radiation. 90% cure rate for stages I and II.

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61
Q

What is the prognosis for brain tumors in children?

A

Second most frequent malignancy in children; mortality 45%. Mortality 45%; also, highest morbidity. More common

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62
Q

What is the most common location for brain tumors in children >1 years old?

A

Supratentorial. Choroid plexus tumors, teratomas

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63
Q

What is the most common location for brain tumors in children 2–10 years old?

A

Infratentorial. Juvenile pilocytic astrocytoma, medulloblastoma (supratentorial)

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64
Q

What is the most common location for brain tumors >10 years?

A

Supratentorial. Diffuse astrocytoma.

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65
Q

What is benign astrocytoma?

A

Most common infratentorial brain tumor. Low–grade, rarely invasive. Most common type of astrocytoma is juvenile pilocytic astrocytoma. Classic site is cerebellum. Surgery, radiation, and chemotherapy. With complete resection, 80–100% survival.

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66
Q

What is malignant astrocytoma?

A

Anaplastic tumor. Glioblastoma multiforme has poor outcome.

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67
Q

What is medulloblastoma?

A

Embryonal tumor. Second most common infratentorial tumor. Mostly males 5–7 years. Midline cerebellar vermis. Solid, homogeneous mass in posterior fossa: ventricular obstruction causes hydrocephalus. Radiation/ chemotherapy result in 70% survival.

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68
Q

What are gliomas?

A

Third most common infratentorial brain tumor. Motor weakness, cranial nerve defects, cerebellar defects, signs of increased ICP. Low–grade gliomas need surgery. Diffuse intrinsic have poor outcome. Radiation:survival 12 mths. Palliative chemotherapy.

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69
Q

What are ependymal tumors?

A

Tumors of the ependymal lining of the ventricles. Most common ependymal tumor is ependymoma. Most are located in the posterior fossa. 10% spread. Well circumscribed, mostly noninvasive. Treatment with surgery, radiation.

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70
Q

What is craniopharyngioma?

A

Most common supratentorial tumor; 10%. Suprasellar. Minimal invasiveness. Calcification. Panhypopituitarism, growth failure, visual loss. Surgery, radiation. Most frequent tumor of optic nerve.

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71
Q

What are the symptoms of craniopharyngeoma?

A

Visual loss, proptosis, eye deviation, optic atrophy, strabismus, nystagmus.

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72
Q

What is the treatment for craniopharyngiomas?

A

Observation. Radiation or chemotherapy if chiasm is involved. Surgery if proptosis with visual loss.

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73
Q

What is a Wilms tumor?

A

Nephroblastoma. Second most common malignant abdominal tumor. Usual age 2–5 years. One or both kidneys (bilateral in 7%).

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74
Q

What is the clinical presentation of Wilms tumor?

A

Asymptomatic abdominal mass

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75
Q

How is Wilms tumor diagnosed?

A

Abdominal CT scan is best test. Chest x–ray for lung involvement.

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76
Q

What is the treatment for Wilms tumor?

A

Surgery, then chemotherapy (vincristine and dactinomycin) and radiation. Bilateral renal: unilateral nephrectomy and partial contralateral nephrectomy.

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77
Q

3–year–old child with bluish skin nodules, periorbital proptosis, periorbital ecchymosis, a hard smooth abdominal mass. What is the diagnosis?

A

Neuroblastoma

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78
Q

What is neuroblastoma?

A

Originates from neural crest cells because of N–myc oncogene; can occur at any site. 8% of childhood malignancies. Most are abdominal, adrenal, retroperitoneal from sympathetic ganglia. 30% are cervical, thoracic, or pelvic ganglia.

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79
Q

What is the presentation of neuroblastoma?

A

Firm, palpable mass in flank or midline; painful; calcification and hemorrhage. Initial presentation often is metastasis to long bones and skull, orbital, bone marrow, lymph nodes, liver, skin.

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80
Q

How is neuroblastoma diagnosed?

A

Plain x–ray, CT scan, MRI. Elevated urine homovanillic acid (HVA) and vanillylmandelic acid (VMA) in 95%. Evaluate for spread with a bone scan, bone marrow biopsy for neuroblasts. Staging: I to IV (disseminated).

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81
Q

What is the treatment for neuroblastoma?

A

Surgery. Chemotherapy (cyclophosphamide and doxorubicin); radiation; bone marrow, or stem cell transplant.

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82
Q

What is a pheochromocytoma?

A

Catecholamine–secreting tumor from chromaffin cells.

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83
Q

What is the most common site of pheochromocytoma?

A

Adrenal medulla, but can occur anywhere along abdominal sympathetic chain; also bladder, urethral walls, thorax, cervical. Children age 6–14 years; more on the right side; >20% are bilateral, and children have multiple tumors.

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84
Q

What diseases are associated with pheochromocytoma?

A

Autosomal dominant; associated with neurofibromatosis, multiple endocrine neoplasias type 2A and MEN2B, tuberous sclerosis, Sturge–Weber syndrome, and ataxia–telangiectasia.

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85
Q

What is the clinical presentation of pheochromocytoma?

A

Episodic HTN, palpitations, diaphoresis, headache, abdominal pain, dizziness, pallor, vomiting, sweating, encephalopathy. Hypermetabolic, polyuria, polydipsia, growth failure. Papilledema, retinal hemorrhages, exudate.

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86
Q

What are the laboratory abnormalities in pheochromocytoma?

A

Significant increase in blood or urinary levels of catecholamines and metabolites

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87
Q

How is pheochromocytoma diagnosed?

A

Most tumors can be localized by CT scan or MRI, but extraadrenal masses are more difficult to fin. 131i–metaiodobenzylguanidine (MBIG) scan detects chromaffin tissue anywhere in body.

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88
Q

Ataxia or opsomyoclonus (dancing eyes dancing feet”) and Horner syndrome are signs of what disease?”

A

Neuroblastoma.

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89
Q

What are the biochemical properties of pheochromocytoma?

A

Children with pheochromocytoma excrete norepinephrine causing increased vanillylmandelic acid and metanephrine. Children with neuroblastoma usually do not have HTN, and major metabolites are dopamine, homovanillyic acid.

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90
Q

What is the treatment for pheochromocytoma?

A

Surgical removal. Preoperative alpha and beta blockade and fluid administration. May manifest later with new tumors.

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91
Q

What is rhabdomyosarcoma?

A

More than 50% are soft–tissue sarcomas. Almost any site. Head and neck–40%. Genitourinary tract–20%. Extremities–20%. Trunk–10%. Retroperitoneal and other–10%. Increased frequency in neurofibromatosis.

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92
Q

What is the clinical presentation of rhabdomyosarcoma?

A

Mass that may be painful. Displacement or destruction of normal tissue. Dissemination to lung and bone.

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93
Q

What is the treatment for rhabdomyosarcoma?

A

Completely remove tumors (but most are not completely resectable). Chemotherapy pre– and postoperatively. Local radiation.

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94
Q

Elevated alpha–fetoprotein is a marker of what disorder?

A

Neural tube defect

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95
Q

What is spina bifida occulta?

A

Midline defect of vertebral bodies without protrusion of neural tissue. Most asymptomatic and of no clinical consequence. Occasionally associated with other anomalies. May have overlying midline lumbosacral defect (hair patch, lipoma, sinus).

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96
Q

What is a tethered cord?

A

Rope–like filum terminale persists and anchors conus below L2. Tension causes asymmetric lower extremity growth, deformities, bladder dysfunction, progressive scoliosis, diffuse pain, motor delay. Midline skin lesion. x–rays show spina bifida; MRI.

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97
Q

What is a meningocele?

A

Meninges herniate through defect in posterior vertebral arches. Fluctuant midline mass covered with skin; may transilluminate. Determine extent of neural involvement with CT scan of head for possible hydrocephalus.

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98
Q

What is the treatment for meningocele?

A

Immediate surgery if there is only a thin cover or leak of CSF.

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99
Q

What are the signs of myelomeningocele?

A

Spinal cord protrudes through an opening along the neuraxis, but most are lumbosacral.

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100
Q

How is myelomeningocele diagnosed?

A

Low sacral lesions: bowel, bladder incontinence, perineal anesthesia. Midlumbar lesion: saclike cyst structure covered by thin tissue. Flaccid paralysis below lesion; no reflexes, no touch, pain. Urinary dribbling, relaxed anus. CT for hydrocephalus.

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101
Q

What preventive treatment reduces the risk of myelomeningocele?

A

Maternal periconceptional use of folate reduces risk of myelomeningocele by half.

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102
Q

What is the treatment for myelomeningocele?

A

Ventriculoperitoneal shunt and correction of defect.

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103
Q

1–month–old infant with head circumference greater than the 95th percentile. What is the diagnosis?

A

Hydrocephalus.

104
Q

What is hydrocephalus?

A

Impaired circulation and absorption of cerebrospinal fluid or, rarely, from increased CSF production from a choroid plexus papilloma.

105
Q

What is obstructive hydrocephalus?

A

Obstruction caused by abnormalities of the cerebral aqueduct (stenosis or gliosis; congenital, intrauterine infection, mumps, hemorrhage) or lesions near the fourth ventricle (brain tumor, Chiari, Dandy–Walker).

106
Q

What are the causes of nonobstructive hydrocephalus?

A

Occurs mostly with subarachnoid hemorrhage; also with pneumococcal or TB meningitis or leukemic infiltrates from obliteration of subarachnoid cisterns or malfunction of arachnoid villi.

107
Q

What is the treatment for all types of hydrocephalus?

A

Ventriculoperitoneal shunting.

108
Q

What is Chiari malformation?

A

Progressive hydrocephalus with myelomeningocele. Abnormality of hindbrain.

109
Q

What is the Dandy–Walker malformation?

A

Cystic expansion of fourth ventricle. Associated agenesis of posterior cerebellar vermis and corpus callosum. Presents with increasing head size and prominent occiput, long–tract signs, cerebellar ataxia, and delayed motor development.

110
Q

What is epilepsy?

A

Epilepsy is present when at least two unprovoked seizures occur more than 24 hours apart.

111
Q

18–month–old child with a generalized, tonic–clonic seizure for 5 min. Cold symptoms with a temperature of 39 C. What is the diagnosis?

A

Febrile seizures

112
Q

What is the age range for febrile seizures?

A

9 months to 5 years of age; peak at 14–18 months. Usually positive family history. Temperature usually increases rapidly to >39 C.

113
Q

What is the presentation of a febrile seizure?

A

Generalized tonic–clonic seizures,

114
Q

What is the prognosis after a simple febrile seizure?

A

Simple febrile seizure is not associated with an increased risk of epilepsy; the risk for febrile seizures is increased with atypical seizure, family history, initial seizure before 9 mths, abnormal development, or preexisting neurologic disorder.

115
Q

What is the evaluation of febrile seizures?

A

Determine cause of fever and rule out meningitis. Labs are not routine needed, no EEG, no neuroimaging. Treatment is control of fever.

116
Q

What is the type I Chiari malformation?

A

Displacement of cerebral tonsils into cervical canal from obstruction of caudal fourth ventricle. Presents in adolescence or adults with recurrent headache and neck pain. Urinary frequency. Progressive spasticity. No hydrocephalus.

117
Q

What are simple partial seizures?

A

Asynchronous tonic or clonic movements of the face, neck, and extremities; average duration 10–20 seconds. May have aura and may verbalize during the attack; no postictal period.

118
Q

What are the electroencephalographic signs of simple partial seizures?

A

EEG spike and sharp waves or multifocal spikes.

119
Q

What is the treatment for simple partial seizures?

A

Phenytoin, carbamazepine; levetiracetam, oxcarbazepine, or zonisamide as adjuncts if needed

120
Q

What are complex partial seizures?

A

Impaired consciousness at some point during the seizures; one–third have aura, which indicates focal onset. Automatisms are common after loss of consciousness and appear as lip–smacking, chewing, swallowing, increased salivation.

121
Q

What are the diagnostic findings in complex partial seizures?

A

Interictal EEG: anterior temporal lobe shows sharp waves or focal spikes. MRI often may show abnormalities in temporal lobe (sclerosis, hamartoma, cyst, infarction, arteriovenous malformation, glioma).

122
Q

What is the treatment for complex partial seizures?

A

Carbamazepine (drug of choice), gabapentin (add–on drug for refractory seizures), lamotrigine (add–on), tiagabine (add–on), topiramate (adjunct for refractory seizures).

123
Q

What is benign centrotemporal (Rolandic) epilepsy?

A

Partial seizure, most often of the face; simple or complex, and may generalize. Repetitive spikes from Rolandic gyri. No neuropathic lesion; excellent prognosis. Occurs at 2–14 years of age. Positive family history.

124
Q

What is the treatment of benign centrotemporal epilepsy?

A

No anticonvulsants unless frequent seizures; carbamazepine is drug of choice; spontaneous remission at 14–16 years of age.

125
Q

What are absence (petit mal) seizures?

A

Sudden cessation of motor activity or speech with blank stare and flickering eyes. More in girls; uncommon

126
Q

What are the ECG findings in absence seizures?

A

EEG shows 3 per second spike and generalized wave discharge.

127
Q

What is the treatment for absence seizures?

A

Ethosuximide (drug of choice), valproic acid.

128
Q

What are tonic–clonic seizures?

A

Aura, loss of consciousness, eyes roll back, tonic contraction, apnea. Then clonic rhythmic contractions alternating with relaxation of all muscle groups. Tongue–biting, loss of bladder control. Semicomatose for 2 h, vomiting, bilateral frontal headache.

129
Q

What is the treatment for absence seizures?

A

Valproic acid, phenobarbital, phenytoin, carbamazepine, gabapentin (add–on), lamotrigine (add–on).

130
Q

What are myoclonic seizures?

A

Repetitive seizures that are brief; symmetric muscle contraction and loss of body tone with falling forward.

131
Q

What is the treatment for myoclonic seizures?

A

Valproic acid and zonisamide (adjunct).

132
Q

What are infantile spasms?

A

Symmetric contractions of neck, trunk, and extremities (with extension).

133
Q

What is the pathophysiology of infantile spasms?

A

Increased corticotropin–releasing factor causes neuronal hyperexcitability. Begin typically at 4–8 months of age.

134
Q

What are cryptogenic infantile spasms?

A

Infant is normal prior to seizure with normal neurologic examination; good prognosis.

135
Q

What are symptomatic infantile spasms?

A

Infantile spasms in an infant with preexisting neurologic disease prior to the seizure (e.g., tuberous sclerosis). Symptomatic infantile spasms are associated with poor seizure control and mental retardation.

136
Q

What are the EEG findings in infantile spasms?

A

Hypsarrhythmia (asynchronous, chaotic bilateral spike–and–wave pattern).

137
Q

What is the treatment for infantile spasms?

A

Adrenocorticotropic hormone (ACTH) is the drug of choice. Prednisone if no response.

138
Q

What are neonatal seizures?

A

Immaturity of CNS. Caused by hypoxic encephalopathy; within 12–14 hours after birth. Other causes include CNS infection, CNS hemorrhage, structural abnormalities, blood chemistry abnormalities, inborn errors, withdrawal.

139
Q

What are the five types of neonatal seizures?

A

Focal (face, extremities), multifocal clonic (many muscle groups), tonic (rigid posturing), myoclonic (brief focal or generalized jerks of extremities), and subtle (chewing, salivation, apnea, blinking, nystagmus, pedaling).

140
Q

What is the laboratory evaluation of neonatal seizures?

A

CBC, electrolytes, calcium, magnesium, phosphorus, glucose, lumbar puncture to exclude meningitis or bleed. EEG normal. Good prognosis. Resolves after 2 years with no treatment.

141
Q

What is the diagnostic evaluation of neonatal seizures?

A

CT scan in term, ultrasound in preterm to diagnose bleed; blood culture and urine culture if fever; newborn screen for inborn errors of metabolism.

142
Q

What is the treatment for neonatal seizures?

A

Lorazepam, phenobarbital.

143
Q

6–year–old with cafe–au–lait lesions, axillary freckling. What is the diagnosis?

A

Neurofibromatosis.

144
Q

What is neurofibromatosis?

A

Von Recklinghausen disease is sometimes inherited in an autosomal dominant manner; but most cases are caused by a new mutation. Every organ can be affected; features at birth, but complications may be delayed into adulthood.

145
Q

What is the diagnostic criteria for neurofibromatosis–1?

A

2 of the following: at least 5 café–au–lait spots >5 mm prepubertal or at least 6 café–au–lait spots >15 mm postpubertal. Axillary, inguinal freckling. >2 iris Lisch nodules (slit lamp). >2 neurofibromas or one plexiform neurofibroma.

146
Q

What are the complications of neurofibromatosis?

A

Gliomas, hamartomas. Malignancy. Cerebral vessel stenosis, aneurysm, TIA, hemiparesis, hemorrhage. Seizures. Learning disabilities, ADHD, speech abnormalities, psychiatric. Renovascular HTN, pheo. Leukemia, rhabdomyosarcoma, Wilms.

147
Q

What is neurofibromatosis–2?

A

Bilateral acoustic neuromas causing hearing loss and facial weakness, headache, unsteady gait, skin findings much less common, CNS tumors common.

148
Q

How is neurofibromatosis–2 diagnosed?

A

One of the following: bilateral acoustic neuromas. Parent, sibling, or child with NF–2 plus unilateral 8th nerve mass; or any two of the following: neurofibroma, glioma, meningioma, schwannoma.

149
Q

What is the treatment for neurofibromatosis–2?

A

Genetic counseling, annual ophthalmologic examination; examine family members.

150
Q

1–month–old infant presents with infantile spasms and a hypsarrhythmic EEG pattern. What is the diagnosis?

A

Tuberous sclerosis.

151
Q

What is tuberous sclerosis?

A

Autosomal dominant; half with new mutations. CNS tubers are found in convolutions of cerebral hemispheres, which undergo calcification and project into ventricular cavity, causing obstruction CSF, hydrocephalus. Retardation.

152
Q

What is the clinical presentation of tuberous sclerosis?

A

Infantile spasms, ash–leaf macule (hypopigmented). Childhood: seizures and skin lesions. Sebaceous adenoma: small red nodules on nose and cheeks. Shagreen patch: rough, raised lesion with orange–peel consistency lumbosacral.

153
Q

How is tuberous sclerosis diagnosed?

A

CT scan shows calcified tubers (but may not be visible until 3–4 years). Renal ultrasound, echocardiogram, chest x–ray.

154
Q

What are the complications of tuberous sclerosis?

A

Retinal mulberry tumor from optic nerve head or phakomas (round, gray lesions). Brain tumors are less common. Half have rhabdomyoma of heart. Renal lesions are common: hamartoma or polycystic kidneys. Pulmonary cystic or fibrous changes.

155
Q

What is Sturge–Weber syndrome ?

A

Facial nevus (port wine stain), seizures, hemiparesis, intracranial calcifications, retardation. Nevus is always present at birth and always involves the upper face and eyelid. Glaucoma in ipsilateral eye. Obtain a skull x–ray and intraocular pressure.

156
Q

What is the presentation of Sturge–Weber syndrome?

A

Seizures in most (focal tonic–clonic, contralateral to the nevus); becomes refractory; hemiparesis, mental retardation.

157
Q

How is Sturge–Weber syndrome diagnosed?

A

Skull x–ray shows occipital–parietal calcifications (serpentine or railroad– track appearance). Increased intraocular pressure. CT scan shows unilateral cortical atrophy and hydrocephalus.

158
Q

What is the treatment for Sturge–Weber syndrome?

A

Control seizures. Hemispherectomy or lobectomy may prevent mental retardation and recalcitrant seizures if done in the first year of life. Regular intraocular pressure evaluation. Nevus is treated with pulsed laser.

159
Q

What are the are four types of cerebral palsy?

A

Spastic dysplasia, spastic quadriplegia, spastic hemiplegia, extrapyramidal.

160
Q

What is cerebral palsy?

A

Group of motor syndromes caused by disorders of early brain development. Most have no cognitive dysfunction; born at term with uncomplicated labor and delivery. Majority are caused by an antenatal problem.

161
Q

What are the signs of cerebral palsy?

A

Impaired voluntary muscle control. Seizures, abnormalities of speech, vision, and intellect. Increased risk with intrapartum infection, low birth weight; most of these caused by intraventricular hemorrhage and periventricular leukomalacia.

162
Q

How is cerebral palsy diagnosed?

A

MRI, hearing and visual evaluation, genetic evaluation.

163
Q

What is the treatment for cerebral palsy?

A

Surgical release procedures, antispasticity drugs (dantrolene, baclofen, botulinum toxin)

164
Q

What is the presentation of the neurodegenerative disorders?

A

Progressive deterioration of neurologic function. Loss of speech, vision, hearing, and walking. Seizures, feeding difficulties, and cognitive dysfunction. Regression of developmental milestones.

165
Q

What is Friedrich ataxia?

A

Neurodegenerative disorder with abnormal gene encoding for frataxin. Autosomal recessive. Onset of ataxia

166
Q

What are the signs of Friedrich ataxia?

A

Absent deep tendon reflexes. Extensor plantar reflex. Weakness in hands and feet. Degeneration of posterior columns: loss of position and vibration. Explosive, dysarthric speech. Kyphoscoliosis. Hypertrophic cardiomyopathy.

167
Q

What is Wilson disease?

A

Inborn error of copper metabolism. Recessive. Liver disease may occur with CNS disease, or liver symptoms may first, neurologic symptoms later. Dystonia, tremors, basal ganglia degeneration. Kayser–Fleischer rings are pathognomonic.

168
Q

What are the MRI abnormalities in Wilson disease?

A

Dilated ventricles with atrophy of cerebrum and lesions in thalamus and basal ganglia.

169
Q

How is Wilson disease diagnosed?

A

Suspect in any child with acute or chronic liver disease, unexplained neurologic disease, or behavioral or psychiatric changes. Best screen is low serum ceruloplasmin. Increased urinary copper excretion. Confirm with liver biopsy, increased copper.

170
Q

What is the treatment for Wilson disease?

A

Chelation with penicillamine. Definitive treatment with liver transplant.

171
Q

What is metachromatic leukodystrophy?

A

Deficiency of arylsulfatase A causes accumulation of cerebroside sulfate in myelin sheath of CNS and peripheral nervous system, resulting in myelin breakdown. An autosomal recessive sphingolipidoses.

172
Q

What are the clinical findings in metachromatic leukodystrophy?

A

Gait disturbance at 1–2 yrs hypotonia, no deep tendon reflexes; can no longer can stand; decreased intellectual; slurred speech; apathetic; decreased vision; nystagmus, optic atrophy. Within year, cannot sit, feed, swallow; decorticate posture.

173
Q

How is metachromatic leukodystrophy diagnosed?

A

Assay of arylsulfatase A. Metachromatic staining of tissue specimens.

174
Q

What is adrenoleukodystrophy?

A

CNS degeneration and adrenal cortical insufficiency. An X–linked recessive sphingolipidoses.

175
Q

What is the presentation of adrenoleukodystrophy?

A

Symptoms at 5–15 y with decreased academic, behavioral changes, gait abnormality, seizures; spasticity, contractures, ataxia, swallowing disturbances; hypoadrenalism, skin hyperpigmentation; deterioration of hearing, vision, sensation; death in 10 y.

176
Q

What is Tay–Sachs disease?

A

Deficient beta–N–acetyl hexosaminidase–A, accumulate GM2. Ashkenazi Jews (carrier rate 1 in 30). Normal development until 6 months, then lag and lose milestones. Seizures, hypotonia, blindness; cherry–red macula.

177
Q

What is Lesch–Nyhan disease?

A

X–linked disorder of purine metabolism, causing excess uric acid. Delayed motor development after a few months. Self–mutilation and dystonia, gouty arthritis. Choreoathetosis, spasticity, renal calculi.

178
Q

How is Lesch–Nyhan disease diagnosed?

A

Presence of dystonia and self–mutilation. Analyze HPRT enzyme.

179
Q

Infant in frog–leg position, with subdiaphragmatic retractions and absent tendon reflexes. What is the diagnosis?

A

Spinal muscle atrophy

180
Q

What is spinal muscle atrophy?

A

Degenerative disease of motor units of the fetus. Denervation of muscle and atrophy. Autosomal recessive.

181
Q

What is the clinical presentation of spinal muscle atrophy?

A

Presents in early infancy with progressive hypotonia, generalized weakness; feeding difficulty. Respiratory insufficiency. Flaccid infant with poor head control. Fasciculation of tongue and fingers. Absent tendon reflexes. Die in first 2 yrs.

182
Q

How is spinal muscle atrophy diagnosed?

A

Molecular genetic marker in blood for the SMN gene. EMG shows fibrillation potential and signs of denervation. Muscle biopsy shows perinatal denervation. Extensive neuronal degeneration and gliosis in anterior horn cells and brain stem motor nuclei.

183
Q

Infant with poor sucking and swallowing, floppy with poor head control. Ocular ptosis and weak muscles on repeated use. What is the diagnosis?

A

Myasthenia gravis

184
Q

What is myasthenia gravis?

A

Immune–mediated neuronal blockade; motor end plate is less responsive because of decreased number of available acetylcholine receptors because of circulating receptor binding antibodies; nonhereditary.

185
Q

What is the clinical presentation of myasthenia gravis?

A

Ptosis and extraocular muscle weakness are earliest findings. Dysphagia and facial weakness; infant feeding difficulties. Poor head control. Limb–girdle, hand weakness. Muscle fatigue late in day. Respiratory muscle involvement.

186
Q

What is transient neonatal myasthenia?

A

Neonates born to mothers with myasthenia have hypotonia, weakness, feeding difficulties, respiratory insufficiency for days. May need ventilation and NG feedings. Infants become normal and have no risk for disease after antibodies wane.

187
Q

How is pediatric myasthenia gravis diagnosed?

A

EMG is more diagnostic than muscle biopsy. EMG shows decremental response to repetitive nerve stimulation, reversed by cholinesterase inhibitor. Anti–acetylcholine antibodies. CPK is normal. Thyroid profile should be checked.

188
Q

What is the treatment for myasthenia gravis?

A

Cholinesterase–inhibitors: neostigmine or pyridostigmine. Severe: long–term prednisone; if no response, IVIg, then plasmapheresis. Thymectomy is most effective if the patient has high anti–ACh titers and symptoms for

189
Q

What are the drug–disease interactions in myasthenia gravis?

A

Neuromuscular blocking agents and aminoglycosides will potentiate symptoms.

190
Q

What is Marie–Charcot–Tooth disease (hereditary motor–sensory neuropathy I)?

A

Progressive disease of peripheral nerves; peroneal muscle atrophy; peroneal and tibial nerve disease. Autosomal dominant.

191
Q

What is the clinical presentation of Marie–Charcot–Tooth disease?

A

Asymptomatic until late childhood. Gait disturbance, fall easily; muscles of anterior, lower leg become wasted. Stork–like appearance. Pes cavus, foot drop, claw hand. Paresthesias. Slowly progressive, but normal lifespan and ambulation.

192
Q

How is Marie–Charcot–Tooth disease diagnosed?

A

Decreased nerve conduction velocities (motor and sensory). CPK is normal. Sural nerve biopsy is diagnostic. Blood molecular genetic diagnosis.

193
Q

What is the treatment of Marie–Charcot–Tooth disease?

A

Surgical ankle fusion; protection from trauma. If paresthesias or pain, phenytoin or carbamazepine.

194
Q

What is Guillain–Barre syndrome?

A

Postinfectious polyneuropathy that is mostly motor; all ages; most with demyelinating neuropathy occurring 10 days after a nonspecific viral illness or Campylobacter jejuni or Mycoplasma pneumoniae.

195
Q

What is the presentation of Guillain–Barre syndrome?

A

Ascending, symmetric paralysis of distal muscles, gradually over days. Pain, paresthesias early. Bulbar involvement, dysphagia, facial weakness, respiratory insufficiency. May have autonomic involvement, BP lability, bradycardia, asystole.

196
Q

What is the disease course in Guillain–Barre syndrome?

A

Spontaneous recovery begins after 2–3 weeks; some have residual weakness.

197
Q

How is Guillain–Barre syndrome diagnosed?

A

Significant increase in CSF protein with normal glucose and no cells. Reduced motor and sensory nerve conduction.

198
Q

What is the treatment for Guillain–Barre syndrome?

A

Hospitalize all patients for observation of respiratory effort. Intravenous immunoglobulin for 2–5 days. May need plasmapheresis, steroids, interferon, immunosuppressives.

199
Q

3–year–old boy with difficulty climbing stairs and frequent falls. Hypertrophy of the calves. What is the diagnosis?

A

Duchenne muscular dystrophy

200
Q

What is Duchenne muscular dystrophy?

A

Primary myopathy X–linked recessive. Progressive degeneration and death of muscle fibers. Most common of the neuromuscular diseases in all races and ethnic groups.

201
Q

What is the clinical presentation of Duchenne muscular dystrophy?

A

Poor head control. By year 2, hip–girdle weakness. Gower sign as early as 3 years of age, but fully developed by age 5–6 years; with hip–waddle gait and lordotic posture. Calf pseudohypertrophy (fat and collagen) and wasting of thigh muscles.

202
Q

What is the disease course of Duchenne muscular dystrophy?

A

Walk until 7 y, then with devices until 12. Second decade with respiratory insufficiency, pulmonary infections, pharyngeal weakness, aspiration, contractures, wheelchair, scoliosis. Cardiomyopathy. Learning disabilities. Death by 18 y.

203
Q

How is Duchenne muscular dystrophy diagnosed?

A

Molecular genetic diagnosis shows a deficiency or defective dystrophin cytoskeletal protein from defective gene. Muscle biopsy shows abnormal or absent dystrophin. CPK is 15,000–35,00. EMG shows myopathic features, but is not specific.

204
Q

What is the treatment for Duchenne muscular dystrophy?

A

Digoxin for heart failure. Antibiotics for pulmonary infections. Calcium supplementation to prevent osteoporosis. Physiotherapy.

205
Q

5–month–old with fever 39 C, hypoactive, decreased appetite. No otitis or focus of infection can be found. What is the diagnosis?

A

Fever without a focus.

206
Q

What is fever without a focus?

A

Fever >38,

207
Q

What are the risk factors for occult bacteremia?

A

38.9 C. Sickle, immunodeficiency, malnourishment. Daycare may carry highly infectious organisms, e.g., N. meningitis, H. influenza. Ill–appearing child, irritable, lethargic, with loss of appetite, petechia.

208
Q

How is fever without a focus diagnosed?

A

Children with WBC counts >15,000 are high risk for bacteremia. Blood cultures. Infants who appear well and have been in good health are not likely to have a serious illness if total WBC is 5,000–15,000 (band count

209
Q

What is the criteria for empiric antibiotic treatment of fever without a focus?

A

Rectal temperature >38 C (>100.4 F). CBC with more than 15,000 WBC with >1,500 bands.

210
Q

What is the criteria for hospitalization of neonates with fever?

A

All neonates (38 C need to be admitted to a hospital for cultures and prophylactic antibiotics to cover for group B Streptococcus, E. coli, and Listeria. Infants >28 d to 3 mths: most common is S pneumoniae.

211
Q

What is the treatment of fever without a focus in infants 28 days to 3 months of age?

A

In the infant who appears well, cultures should be obtained. Treat the patient with IM ceftriaxone once. Follow up in 24 hours. In the infant who appears toxic: admit and obtain cultures first, then start empiric IV antibiotics.

212
Q

What is the treatment of fever without a focus in infants >3 months to 3 years?

A

In toxic appearing patients, admit for IV antibiotics after obtaining blood cultures. If >39 C and the child is not toxic appearing, obtain cultures, treat with IM ceftriaxone, and return in 24 h. If

213
Q

What is fever of unknown origin?

A

FUO is a documented fever with no cause after 3 weeks of evaluation on an outpatient basis or 1 week in hospital.

214
Q

6–year–old with headache, vomiting, neck stiffness, and photophobia. Ill–appearing child; neck pain with flexion. Kernig and Brudzinski signs are positive. What is the diagnosis?

A

Meningitis

215
Q

What are the causes of bacterial meningitis in the first 2 months of life?

A

Maternal vaginal flora: group B Streptococcus, E. coli, Listeria.

216
Q

What are the causes of bacterial meningitis age 2 months to 12 years?

A

S. pneumoniae (peaks in first 2 years), N. meningitidis (sporadic or in epidemics; direct contact from a daycare center or adult family member; increased in college freshmen living in dorms).

217
Q

What is the pathophysiology of bacterial meningitis?

A

Meningeal inflammation and exudate. Most from hematogenous spread, initially from bacterial colonization of nasopharynx. Rarely from an infection at a contiguous site, such as sinusitis, otitis media, mastoiditis, orbital cellulitis.

218
Q

What is the clinical presentation of bacterial meningitis?

A

Less commonly a dramatic, sudden, rapid deterioration over hours to a day, with shock and death. More commonly there are several days with fever, lethargy, irritability, anorexia, nausea, vomiting. Meningeal irritation (photophobia, neck pain, rigidity).

219
Q

What is Kernig sign?

A

Flexing of the hip 90 degrees causes pain with leg extension. Not present in infants.

220
Q

What is Brudzinski sign?

A

Involuntary flexing of knees and hips after passive flexing of the neck while supine. Not present in infants.

221
Q

What are the signs of increased intracranial pressure?

A

Headache, emesis, bulging anterior fontanelles, oculomotor or abducens palsies, hypertension with bradycardia, apnea, decorticate or decerebrate posturing, stupor, coma.

222
Q

How is meningitis diagnosed?

A

Lumbar puncture and blood culture in all (90% have a positive blood culture).

223
Q

What are contraindications to immediate lumbar puncture?

A

Evidence of increased intracranial pressure. Severe cardiopulmonary problems requiring resuscitation. Infection of skin over site. Do not delay antibiotics while waiting for CT scan.

224
Q

What are the CSF findings in pyogenic meningitis?

A

200–5,000 cells/mL, PMNs, low glucose, high protein; positive Gram stain; positive culture, positive counter immunoelectrophoresis or latex agglutination for pneumococcal antigen; high CSF pressure.

225
Q

What are the CSF findings in partially treated meningitis?

A

200–5,000 cells/mL, mostly PMNs, low glucose, high protein, variable Gram stain, variable culture; positive counter immunoelectrophoresis or latex agglutination for pneumococcal antigen; high CSF pressure.

226
Q

What are the CSF findings in granulomatous meningitis?

A

100–500 cells/mL, lymphocytes, low glucose, high protein; negative Gram stain, positive culture; negative CIE or LA; high CSF pressure.

227
Q

What are the CSF findings in aseptic meningitis?

A

100–700 cells/mL; PMN, lymphocytes; normal glucose, slightly high protein, negative Gram stain, negative culture, negative CIE or LA; normal CSF pressure.

228
Q

What are the CSF findings in a neighborhood reaction?

A

100–500 cells/mL, variable cytology, normal glucose, variable protein; negative Gram stain, negative culture, variable CIE or LA, variable pressure.

229
Q

What is a CSF neighborhood reaction?

A

A localized focus of infection near the subarachnoid space may express some products of inflammation into the CSF. Neighborhood infections are brain abscesses, an epidural abscesses, sinusitis, or mastoiditis.

230
Q

What is the normal CSF glucose concentration?

A

CSF glucose is 50–70% of blood glucose. CSF glucose concentration should be considered in relation to blood glucose concentration.

231
Q

What is the treatment for meningitis?

A

Initial empiric treatment of meningitis is vancomycin and either cefotaxime or ceftriaxone.

232
Q

What is specific treatment of S pneumoniae meningitis?

A

Penicillin or third–generation cephalosporin for 10–14 days.

233
Q

What is specific treatment of Neisseria meningitidis meningitis?

A

Penicillin for 5–7 days. HiB: ampicillin for 7–10 days.

234
Q

What is specific treatment of meningitis that has been pretreated and no organism is identified?

A

Third–generation cephalosporin for 7–10 days.

235
Q

What is the treatment of Gram–negative (E. coli) meningitis?

A

Third–generation cephalosporin for 3 weeks, or for at least 2 weeks after sterilization.

236
Q

What is the indication for adjunctive dexamethasone in the treatment of meningitis?

A

Dexamethasone is added to initial treatment of meningitis caused by Haemophilus influenzae type B because it decreases the incidence of fever, elevated CSF protein, and 8th cranial nerve damage (hearing loss).

237
Q

What are the complications of meningitis?

A

Increased ICP with herniation, seizures. Subdural effusion. CN palsies, stroke, thrombosis of dural venous sinuses. Most commonly hearing loss (pneumococcus). Retardation, developmental delay, visual impairment.

238
Q

What is the prevention of bacterial meningitis?

A

Chemoprophylaxis with rifampin for N. meningitidis and HiB, but not for S. pneumoniae. Prophylaxis for all close contacts regardless of age or immune status.

239
Q

What is the presentation of acute meningococcemia?

A

Initially fever, flu–like symptoms. Meningococcal rash before more serious signs develop. Rapid progression to septic shock, DIC, acidosis, adrenal hemorrhage, renal and heart failure. Any organ can be affected by vasculitis, thromboembolism.

240
Q

What is purpura fulminans?

A

Petechiae, purpura and meningitis.

241
Q

What is viral (aseptic) meningitis?

A

Viral infection of the meninges and brain tissue; usually self–limited; spread by person–to–person contact in summer and fall, usually enteroviruses.

242
Q

What is the mode of transmission of arbovirus encephalitis?

A

Arbovirus is an arthropod–borne viruses; vectors are mosquitoes and ticks after biting infected birds or small animals; spreads to humans and other vertebrates. Rural exposure more common.

243
Q

What is the presentation of herpes simplex encephalitis?

A

Fever (90%), headache (81%), psychiatric symptoms (71%), seizures (67%), vomiting (46%), focal weakness (33%), memory loss (24%); progresses to coma and death without treatment.

244
Q

What is the presentation of varicella zoster encephalitis?

A

Most common presentation is cerebellar ataxia and acute encephalitis.

245
Q

Which patients are at risk for of cytomegalovirus meningitis?

A

In immunocompromised, disseminated disease; or congenital infection.

246
Q

What is the clinical presentation of aseptic meningitis?

A

Headache and hyperesthesia in older children. Irritability and lethargy in infants. Fever, nausea, vomiting, photophobia, and neck, back, and leg pain. Exanthems, especially with echovirus and Coxsackie, varicella, measles, and rubella.

247
Q

What are the complications of aseptic meningitis?

A

Guillain–Barre syndrome, transverse myelitis, hemiplegia, cerebellar ataxia. Most completely resolve without problems except that neonates with HSV have severe sequelae.

248
Q

How is aseptic meningitis diagnosed?

A

PCR of CSF is the best test. Viral culture.

249
Q

What is the treatment for aseptic meningitis?

A

Acyclovir is indicated for herpes simplex virus (HSV).

250
Q

What type of encephalitis commonly affects the temporal lobe?

A

Herpes simplex virus encephalitis. Focal seizures, CT scan or MRI abnormalities, and EEG findings are highly suspicious for herpes simplex virus encephalitis.

251
Q

What are the signs of encephalitis?

A

Meningitis plus mental status changes.

252
Q

11–month–old unimmunized child, with paroxysmal cough, fever. Facial petechiae and conjunctival hemorrhages. What is the diagnosis?

A

Pertussis

253
Q

What is the mode of transmission of Bordetella pertussis?

A

Endemic and very contagious by aerosol droplets. Neither natural disease nor vaccination provides complete or lifelong immunity; immunity wanes after 3–5 years. Coughing adolescents and adults are reservoirs.

254
Q

What is the clinical presentation of whooping cough?

A

Catarrhal phase (2 wks) with cold symptoms. Paroxysmal phase (2 wks) with increasing to severe coughing paroxysms, inspiratory whoop and facial petechiae. Convalescent phase lasts 2 weeks with gradual resolution.

255
Q

How is pertussis infection diagnosed?

A

History of incomplete immunizations. Gold standard is culture (nasopharyngeal aspirate or cough). PCR or direct fluorescent antibody test may be helpful if there has been antibiotic usage. Leukocytosis, lymphocytosis.

256
Q

What is the treatment for whopping cough?

A

Always treat suspected cases with erythromycin for 14 days or other macrolides. Treatment decreases infectious period of patient and may shorten the course of illness; also treat all household members and any close contacts.

257
Q

How is Bartonella henselae infection diagnosed?

A

Most common cause of lymphadenitis lasting more than 3 weeks. Cutaneous inoculation by arthropod borne by cat flea; kittens transmit better than cats. Incubation period is 3–30 days.

USMLE Pediatrics (4 decks)

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