Neuromuscular

Home, Search, Index, Links, Pathology, Molecules, Syndromes,
Muscle, NMJ, Nerve, Spinal, Ataxia, Antibody & Biopsy, Patient Info


NEUROMUSCULAR DISORDERS & ENDOCRINE DISEASE

Acromegaly
Adrenal
Carcinoid myopathy
Corticosteroid
Diabetes
Gonadal
  Dysgenesis
  Gynecomastia
  Hypogonadism
Insulinoma
Parathyroid
  Hyperparathyroid
  Hypoparathyroid
Thyroid
  Hyperthyroid
  Hypothyroid

From Bramwell: Atlas of Clinical Medicine

Myxedema




THYROID DISEASE

Hyperthyroid
  Myopathy
  Ophthalmopathy
  Other associated disorders
Hypothyroid
  Adult
  Childhood
  Other associated disorders

External link: Testing

From Bramwell: Atlas of Clinical Medicine


Hypothyroidism


Oppenheim 1894

Dercum 1894

Hyperthyroidism


From T Miller

Hyperthyroid


Hyperthyroid after treatment

Booth 1894


PARATHYROID DISEASE

Hypoparathyroidism



Hyperparathyroidism


ADRENAL & CORTICOSTEROIDS

Adrenal insufficiency
Corticosteroid-binding globulin deficiency
Corticosteroid myopathy
Corticosteroid withdrawal
Myosin loss myopathy
Nelson

Cushing 1912
Cushing's Syndrome
("Pluriglandular Syndrome")
Corticosteroid Potencies
Drug Potency
Gluco- Mineralo-
SHORT-ACTING
Hydrocortisone 1 1
Cortisone 0.8 0.8
INTERMEDIATE-ACTING
Prednisone 4 0.25
Prednisolone 4 0.25
Methylprednisolone 5 <0.01
Deflazacort 5.2 ?
Triamcinolone 5 <0.01
LONG-ACTING
Paramethasone 10 <0.01
Betamethasone 25 <0.01
Dexamethasone 30–40 <0.01

Corticosteroid myopathy
  • Precipitants
    • Drug-induced
      • Especially with 9-α-fluorinated corticosteroids
      • Rare with Prednisone, especially < 30 mg qd
      • Also see: Myosin-loss myopathies
    • Endogenous
      • Cushing's syndrome 11
        • 50% to 80% of cases
        • General clinical features: Each may only occur in minority of patients
          • Fat distribution
            • Abnormal in supraclavicular & temporal fossae
            • Facial rounding ("moon faces")
            • Obesity
          • Muscle weakness: Proximal
          • Skin
            • Purple striae: Wide (> 1 cm)
            • Bruising
            • Hirsutism
            • Wound healing: Poor
          • Linear growth in children
            • Decreased with continued weight gain
          • Behavioral
            • Decreased libido: 91%-100%
            • Psychiatric: Lethargy, Depression
          • Other endocrine
            • Impaired glucose tolerance/diabetes
            • Menstrual changes
          • Atherosclerosis
        • Diagnosis
          • Cortisol
            • 24 hour urine free cortisol
            • Salivary cortisol
          • Dexamethasone suppression test
          • ACTH measurement
        • Rule out
          • Low K+
          • Low PO4
        • Treatment
          • Optimal: Surgical removal of tumor
          • Medical: Ketoconazole
      • Ectopic ACTH production
  • Clinical
    • Muscle
      • Weakness: Proximal; Symmetric; Legs > Arms
      • Wasting
      • Myalgias
    • Systemic
      • Weight gain (90%)
      • Hypertension (85%)
      • Skin: Hirsutism; Fragile; Plethora
      • Osteopenia
      • Neuropsychiatric
      • Metabolic: Glucose intolerance (75%); Diabetes (20%); Hyperlipidemia (70%)
      • Gonadal dysfunction: Menstrual change (70%); Impotence (85%)
  • Lab
  • Mechanisms
    • Muscle protein catabolism: Increased
    • ? Other
  • Treatment: Reduce steroid dose

From Pincus Taft
Cushing's Syndrome

From Pincus Taft

Myosin loss myopathy


Nelson's syndrome: After total bilateral adrenalectomy
Adrenal insufficiency

From Bramwell: Atlas of Clinical Medicine

Addison's Disease

Fatigue syndrome with corticosteroid-binding globulin deficiency

  SERPINA6 ; Chromosome 14q32.1; Dominant
Corticosteroid withdrawal

ACROMEGALY
Cushing: The pituitary body & its disorders

Before disease

Disease onset

12 yrs of disease

17 yrs of disease

Square hand


Phalanges:
Tufting; Exostoses


Putnam

ACROMEGALY




CALCIPHYLAXIS 6



Hypoglycemic Neuropathy 5



Gynecomastia & Neuromuscular


Gonadal Disorders



Return to Neuromuscular Home Page

References
1. JNNP 2000;68:681-2
2. Ann Int Med 2000;133:197-201
3. Semin Neurol 2000;20:43-54
4. Muscle Nerve 2000;23:1891-1894
5. Acta Neuropathol 2001 (On-line)
6. Lancet 2001;358:1115-1116; Kidney Int 2001; 60:324-332
7. Eur J Neurol 2003;10:87-90
8. Ann Intern Med 2005;142:310
9. Arq Neuropsiquiatr 2004;62:154-157
10. Endocr Res 2005;31:171-175,
11. American Journal of Medicine 2005;118:1340-1346, NEJM 2017;376;1451-1459

4/13/2017