Updated: 4/12/2019

Tourette Syndrome

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Snapshot
  • A 6-year-old boy is brought to the pediatrician by his mother due to noticing him "acting strange." She reports that the patient would jerk his head rapidly and sniff a considerable amount of times per day for over a year. The jerking and sniffing episodes would begin and end abruptly, and the frequency would increase during periods of increased stress. She notices the patient trying to suppress these sniffing episodes and jerking movements, and appears relieved after they occur. Neurologic exam is normal besides the neck jerking and sniffing. 
Introduction
  • Clinical definition
    • a chronic neurologic disorder that manifests with motor and vocal tics
  • Epidemiology
    • incidence
      • variable
    • demographics
      • more common in males (4:1)
      • mean age of tic onset is approximately 5.6 years of age
  • Pathogenesis
    • the mechanism of disease is unclear; however, it is believed to be due to a complex interaction between genetic, environmental, and social factors resulting in
      • an abnormality in the mesolimbic spinal system
  • Genetics
    • inheritance pattern
      • unclear
    • mutations
      • there are several genes that may be associated with Tourette syndrome (TS)
  • Associated conditions
    • approximately 90% of patients have a comorbid psychiatric disorder such as
      • attention deficit hyperactivity disorder (~60% of cases)
      • obsessive-compulsive disorder (~27% of cases) 
  • Prognosis
    • tics typically decline during adolescence and may
      • resolve around 18 years of age (~50% of children)
    • tics may persist into adulthood but their severity can decline over time
Presentation
  • Symptoms and physical exam
    • tics (hallmark of TS)
      • have a sudden onset and of brief duration that can be
        • motor (e.g., eye blinking, body gyrations, and head jerking)
        • vocal (e.g., simple noises, coprolalia, and palilalia)
      • waxing and waning nature
      • urge before the tic that is relieved after the tic
    • neurologic exam is typically normal besides for tics
Studies
  • Diagnostic criteria
    • according to the DSM V
      • both multiple motor and ≥ 1 vocal tic
        • this does not necessarily have to occur concurrently
      • tics persist ≥ 1 year since first tic
        • tics may wax and wane in frequency
      • tic begin at < 18 years of age
      • the tics are not due to other causes (e.g., substance use and medical conditions such as Huntington's disease)
Differential
  • Transient motor and phonic tics which lasts
    • < 1 year
  • Myoclonus
  • Dystonia
  • Chorea
  • Stereotypies
Treatment
  • Conservative
    • behavioral therapy
      • indication
        • can be considered in patients with TS who have moderate tics, tics that cause impairment, or when comorbid psychiatric conditions that respond to behavioral therapy are present
      • modalities
        • habit reversal training
  • Medical
    • tetrabenazine
      • indication
        • used for the pharmacologic treatment of TS
    • α-2 adrenergic agonists
      • medications
        • clonidine
        • guanfacine
      • indication
        • used for the pharmacologic treatment of TS
    • atypical antipsychotics
      • medications
        • risperidone
        • olanzapine
        • quetiapine
        • ziprasidone
        • aripiprazole
      • indication
        • used for the pharmacologic treatment of TS
  • Operative
    • deep brain stimulation
      • indication
        • can be considered in patients with TS who are refractory to optimal medical therapy; however,
          • larger clinical trial are needed
Complications
  • A reduction in quality of life
  • May develop depression
 

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Questions (1)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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