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CPC 17 th April 2008. Female, 48 years old 2 years prior to admission: Proximal muscle weakness left lower extremity 1.5 years prior to admission:Weakness, on wheel chair Muscle atrophy 1 year prior to admission :Upper extremities weakness - PowerPoint PPT Presentation
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CPC 17th April 2008
Female, 48 years old
2 years prior to admission: Proximal muscle weakness left lower extremity
1.5 years prior to admission: Weakness, on wheel chair Muscle atrophy
1 year prior to admission : Upper extremities weakness
1 week prior to admission : Quadriparesis
MRI: At 1.5 years prior to admission(lower extremities weakness with muscle atrophy)Cervical spondylosis, cervical canal stenosis C5-T1Herniated disc C 3-7
Physical therapy/ Orthropaedic surgery: C3-4 with instrument
Asymptomatic Degenerative Disk Disease andSpondylosis of the Cervical Spine: MR Imaging’
-1987 1648388Radiology ; :
<40 Yrs. Old (N = 167) >40Yrs. Old (N= 97)
Major Minor Major Minor Abnormality Abnormality Abnormality Abnormality
Herniated disc 5 (3%) 7 (4%) 1 (1%) 4 (4%)Bulging disc 0 8 (5%) 1 (1%) 5 (5%)Foraminal stenosis 5 (3%) 7 (4%) 9 (9%) 14 (14%) Disc-space narrowing 3 (2%) 18 (1 1%) 15 (16%)
21 (22%)Degenerated disc 13 (8%) NA 36 (37%) NA Spurs (spondylosis) 5 (3%) 23 (14%) 6 (6%) 33 (34%) Abnormal cords 15 (9%) 15 (9%) 1 (1%) 17 (18%)
Abnormal magnetic-resonance scans of the cervical spine in asymptomatic subjects. A prospective investigation
J Bone Joint Surg Am. 1990;72:1178-1184.
• Muscle weakness/motor predominate
• No cranial nerves involvement
• No paresthesia
• No bowel/urinary bladder/autonomic dysfunction
• Asymmetry to symmetry by clinical course
• Chronic progressive course
• Muscle atrophy
Corticospinal tract
Anterior horn cell: Amyotrophic lateral sclerosis,
Radiculoneuropathy: Demyelination disease
Mononeuritis multiplex
Neuromuscular junction: Myasthenia gravisLambert-Eaton myasthenic syndrome
Myopathy: Polymyositis, Metabolic disorder
•Cervical spondylosis•Compressed fracture C6•HNP C 3-7, •Kyphoscoliosis•Osteopenia
Metabolic Bone DiseaseMetabolic Bone Disease
Bone turnover: Primary hyperparathyroidism Secondary hyperparathyroidism
Adynamic bone disease
Low Bone content: Osteoporosis
High bone content: Osteopetrosis Bisphosphonate
Abnormal mineralization: Osteomalacia/rickets
Causes of OsteomalaciaCauses of Osteomalacia Abnormal matrixAbnormal matrix
Abnormal vitamin D metabolismAbnormal vitamin D metabolismGastrointestinal diseaseGastrointestinal diseaseChronic liver diseaseChronic liver diseaseChronic kidney diseaseChronic kidney diseaseHypoparathyroidismHypoparathyroidism
Mineralization inhibitorMineralization inhibitorAluninum toxicityAluninum toxicityFluorideFluorideChronic metabolic acidosisChronic metabolic acidosis
Hypophosphatasia (AR)Hypophosphatasia (AR)
HypophosphatemiaHypophosphatemia
•Labs:
•BUN 9 mg %, Cr 0.4 mg %,
•Calculated creatinine clearance 151 mL/min
•Measured creatinine clearance 68.2 mL/min
•Na+ 142 mEq/L, K+ 3.7 mEq/L, Cl- 105 mEq/L, HCO3- 27 mEq/L
•Ca2+ 10.4 mg%, PO42- 1.3 mg%
•iPTH 72 pg/mL
Daily requirement = 800 mg Foods
Meat, poultry & fish Dairy products Processed foods Soda
•Etiology of Hypophosphatemia
•Internal redistribution•Re-feeding•Acute respiratory alkalosis•Hungry bone syndrome
•Decreased intestinal absorption•Inadequate intake (< 100 mg/day)•Chronic diarrhea, malabsorption•Vitamin D deficiency or resistance•Aluminum or magnesium ingestion
•Increased urinary excretion•Primary hyperparathyroidism•Secondary hyperparathyroidism•Proximal tubule dysfunction•Hereditary hypophosphatemic rickets•Onchogenic osteomalacia
Filtered Phosphate
Excreted Phosphate
Renal phosphate wasting
• 24 hours urine phosphate = 787.4 mg/day ( < 100 mg/day)
• Fractional PO42+ excretion = U PO
42+ xPcr
P PO4
2+ xUcr = 26.73% (5 -10 %)
• Renal phosphate clearance = U PO42+ x V
P PO4
2+ x 1440
= 27.34 mL/min (5 -15 % mL/min)
Renal threshold phosphate conc (Tm PO4
2+/GFR)
Normal 2.0 – 3.5 mg% = 0.9 mg%
Lancet1975;309-10
.. GlucosuriaGlucosuria HypophosphatemiaHypophosphatemia HypouricemiaHypouricemia HypokalemiaHypokalemia AminoaciduiraAminoaciduira
Autosomal dominant, recessive, or X-linkedAutosomal dominant, recessive, or X-linked Wilson’s disease, Wilson’s disease, Galactosemia, tyrosinemia, cystinosis Galactosemia, tyrosinemia, cystinosis Multiple myeloma, amyloid, Multiple myeloma, amyloid, Heavy metal toxicity, chemotherapeutic drugs Heavy metal toxicity, chemotherapeutic drugs
(ifosfamide), imatinib mesylate(ifosfamide), imatinib mesylate
Disorder Serum phosphate
Serum calcium ALP
Vit D deficiency Low Low Elevated
Renal phosphate wasting Low Normal Normal
Chronic metabolic acidosis Normal Normal Normal
Proximal RTA Low Normal Normal
Hypophosphatasia Normal Normal Low
Bone matrix abnormality Normal Normal Normal
Hypophosphatemia with renal phosphate wasting
• Proximal RTA
• Hyperparathyroidism
• Mutation of type 2a sodium-phosphate co-transport: Absorptive hypercalciuria type III, nephrolithiasis
Hereditary hypophosphatemic rickets with hypercalciuria
• Hereditary hypophosphatemic ricket
• Tertiary hyperparathyroidism post-kidney transplantation
• Onchogenic osteomalacia: High phosphatonin: FGF 23, matrix extracellular phosphaglycoprotein (MEPE), frizzled growth factor 4 (FRP-4)
iPTH stimulate FGF 23
Kidney Int 2003, 64 : 2272–2279
Log FGF-23
CKD, Renal bone disease, Aging
New Engl J Med 2003;348:1656
•Fibrous dysplasia
•Hemangiopericytoma
•Osteosarcoma
•Chrondroblastoma
•Chondromyxoid fibroma
•Malignant fibrous histiocytoma
•Giant cell tumor
•hemangioma
Mayo clinic 1994
Mesenchymal tumor cause TIO
•Progressive muscle weakness•Severe metabolic bone disease/Osteomalacia•Hypophosphatemia/Severe renal phosphate wasting•Groin mass
Onchogenic osteomalacia/immobilization osteoporosis
Investigation: Groin mass excision/immunohistochem staining Serum FGF-23 concentration (Octreotide labeled indium-111 scan)
Mesenchymal tumor mass
Acquired high phosphatonin (FGF-23)
Severe renal phosphate wasting
Severe osteomalacia, immobilization osteopenia
Muscle weakness
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