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Periodic Paralysis
Definition
PATHOPHYSIOLOGY
Total body deficit
of potassium
Acute potassium
depletion
diabetic ketoacidosis,
severe GI losses : vomiting / diarrhea,
dialysis, and diuretic therapy
potassium shifts
from the EC
to IC space
Other causes
S. K+ level
Clinical features
<3.5 mmol/l
Lassitude
<2.5mmol/l
<2 mmol/l
Definition
Effects of hypokalemia
Atrial/ventricular
life-threatening
Effects of hypokalemia
severe
Effects of hypokalemia
hypokalemia
hypokalemia
hypokalemia
Investigations
Although
Investigations
Drug
screen (serum or
urine):
Amphetamines
and other
sympathomimetic
stimulants can cause
hypokalemia.
Other
drugs include
verapamil
overdose.
Theophylline.
amphotericin
B.
Aminoglycosides.
cisplatin.
Hormonal assay:
Serum ACTH,
Cortisol,
Renin activity,
Aldosterone
Hypokalemic
Periodic Paralysis
(HypoPP)
onset of disease:
childhood or puberty
clinical features:
weakness episodes:
Provocative factors:
between episodes:
etiology:
Na+ CHANNELOPATHIES
Na+ CHANNELOPATHIES
clinical features
Hypokalemia potentiates the effects of digoxin and may cause digitalis intoxication
ECG changes :
Management
Periodic Paralysis
HypoKPP
1 msec
Muscle channelopathies
Non
dystrophic myotonias
Myotonia
congenita (CLCN1)
Paramyotonia
congenita (SCN4A)
Sodium
Periodic
paralyses
Hypokalemic
Hyperkalemic
Anderson
(CACNA1S/ SCN4A)
(SCN4A)
periodic paralysis
hyperaldosteronism
RTA
villous adenoma
cocaine binge
diuretics, licorice, steroids, ETOH
Hyperkalemic
(k>7):
hyporenemic
hypoaldosteronism (DM/CRF)
oral K, CRF, chronic heparin, rhabdomyolysis
Normakalemic:
Guanidine,
29
in 100,000
Autosomal
M:F
3 or 4:1
Onset:
30
Clinical features
Flaccid
Occur
Absence
of myotonia
Proximal
Sparing
Lasts
Frequency:
highly variable
Frequency
Permanent
Attacks
Precipitating factors
Strenuous
High
carb diet
ETOH
consumption
Emotional
stress
Concurrent
Lack
viral illness
of sleep
Medications
Arg528His,
Arg1239His, Arg1239Gly
Less
34
Reduced
SER
Reduced
Impaired
E-C coupling
Ca
Diagnostic studies
Serum
K < 3.0mEq/L
Serum
CK level elevated
EKG
Provocative
load/ insulin
Electrophysiology
Sensory
attacks
During
Periodic Paralysis
Muscle Pathology
Muscle
Vacuoles
Vacuoles
represent proliferation,
degeneration and autophagic destruction
of T-tubules & SR
Large
38
Treatment
Reducing
Acute
treatment replacement of K
Acetazolamide
prevent attack
recurrence and severity
Acetazolamide
HypoKPP2
Dichlorphenamide
no longer
available
Triamterene
and spironolactone
39
Hypo: gluc/insulin
Hyper: K+
Electrophysiology
EMG
Genetics
40
useful in MC
Baseline
CMAP
Exercise
10 sec
Record
PMC
exacerbated by cold
HypoKPP
41
Record
42
References
Anthony A.Amato and James A.Russell; Non dystrophic myotonias and periodic
paralysis. Neuromuscular disorders 2008, Mc Graw Hill, Section II Chapter 29; 655680
Burge JA, Hanna MG. Novel insights into the pathomechanisms of skeletal muscle
channelopathies; Curr Neurol Neurosci Rep. 2012 Feb Vol 12:62-69
neuromuscular.wustl.edu
43
Disease characteristics