Periodic Paralyses Differential Diagnoses
- Author: Naganand Sripathi, MD; Chief Editor: Nicholas Lorenzo, MD more...
Differential Diagnoses
- Acute Inflammatory Demyelinating Polyradiculoneuropathy
- Cauda Equina and Conus Medullaris Syndromes
- Chronic Inflammatory Demyelinating Polyradiculoneuropathy
- Guillain-Barre Syndrome in Childhood
- Lambert-Eaton Myasthenic Syndrome
- Multiple Sclerosis
- Myasthenia Gravis
- Spinal Cord Hemorrhage
- Spinal Cord Infarction
- Spinal Cord, Topographical and Functional Anatomy
- Spinal Epidural Abscess
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- Table 1. Primary Periodic Paralysis
- Table 2. Distinguishing Features Among the Common Forms of Periodic Paralyses
- Table 3. Differential Diagnosis of Secondary Periodic Paralyses
- Table 4. Differential Diagnosis of Other Entities Causing Acute Generalized Weakness
- Table 5. Medical Conditions Associated With Hypokalemia
- Table 6. Diagnostic Studies of Hypokalemic and Hyperkalemic Periodic Paralyses
- Table 7. Electrophysiological Patterns to Exercise Testing
| Sodium channel | Hyperkalemic PP (HyperPP) Hypokalemic PP (HypoPP2) Paramyotonia congenita |
| Calcium channel | Hypokalemic PP (HypoPP1) |
| Potassium channel | Andersen-Tawil syndrome Hyperkalemic PP or hypokalemic PP* |
| *The deficit was described in 2 small families and has not been substantiated by others.[1, 2] | |
| Syndrome | Age of Onset | Duration of Attack | Precipitating Factors | Severity of Attacks | Associated Features |
| Hyper-kalemic periodic paralyses | First decade of life | Few minutes to less than 2 h (mostly less than 1 h) | Low carbohydrate intake (fasting) Cold Rest following exercise Alcohol Infection Emotional stress Trauma Menstrual period | Rarely severe | Perioral and limb paresthesias Myotonia frequent Occasional pseudo-hypertrophy of muscles |
| Hypo-kalemic periodic paralyses | Variable -Childhood to third decade Majority of cases before 16 years | Few hours to almost a week Typically no longer than 72 h | Early morning attacks after previous day physical activity High-carbohydrate meal, Chinese food, alcohol Cold, change in barometric pressure or humidity Fever, upper respiratory tract infections Lack of sleep, fatigue Menstrual cycle | Severe Complete paralysis | Occasional myotonic lid lag Myotonia between attacks rare Unilateral, partial, monomelic Fixed muscle weakness late in disease |
| Potassium- associated myotonia | First decade | No weakness | Cold Rest after exercise | Attacks of stiffness can be mild to severe | Muscle hypertrophy |
| Para-myotonia congenita | First decade | 2-24 h | Cold | Rarely severe | Pseudo-hypertrophy of muscles Paradoxical myotonia Fixed weakness rare |
| Thyrotoxic periodic paralyses | Third and fourth decades | Few hours to 7 d | Same as hypokalemic PP Hyper-insulinemia | Same as hypokalemic PP | Fixed muscle weakness may develop Hypokalemia during attacks |
| Hypokalemic | Hyperkalemic |
Urinary potassium-wasting syndromes
| |
| Alcohol | Addison disease Chronic renal failure Hyporeninemic Hypoaldosteronism |
| Drugs - Amphotericin B, barium | Ileostomy with tight stoma |
| Renal tubular acidosis | Potassium load |
GI potassium-wasting syndromes
| Potassium-sparing diuretics |
| Disorder | Pattern and Distribution of Weakness |
| Transient ischemic attacks | Follow CNS distribution (ie, hemiparetic) May have sensory symptoms and signs |
| Sleep attacks | Occur at onset or termination of sleep Last only minutes |
Myelopathy
| Sensory symptoms Presence of a sensory level Sphincter involvement |
| Myasthenia gravis Lambert-Eaton myasthenic syndrome | Subacute in onset Associated autonomic symptoms in LEMS Hyporeflexia in LEMS Abnormal repetitive nerve stimulation Presence of distinct antibodies |
Peripheral neuropathy of acute onset
| Pattern of weakness Absent stretch reflexes |
Toxins
| Clinical presentation |
| Urine K/C Ratio | Acid Base Status | Other Associated Features | Medical Conditions |
| < 1.5 | Metabolic acidosis | Lower GI loss – Laxative abuse, diarrhea | |
| < 1.5 | Metabolic alkalosis | Normal BP | Surreptitious vomiting |
| >1.5 | Metabolic acidosis | DKA, type 1 or type 2 distal RTA | |
| >1.5 | Metabolic alkalosis | Normal BP | Diuretic use, Bartter syndrome, Gitelman syndrome |
| ≥1.5 | Metabolic alkalosis | Hypertension | Primary aldosteronism, Cushing syndrome, renal artery stenosis, congenital adrenal hyperplasia, apparent mineralocorticoid excess, Liddle syndrome |
| Hypokalemic PP | Hyperkalemic PP | |
| Serum potassium | Mildly depressed; may reach 1-5 mEq/L | Increases from baseline but may not increase beyond normal range |
| Serum CPK | Moderately elevated during attacks | Mildly elevated during attacks |
| ECG | Bradycardia Flat T waves, U waves, ST-segment depression | Tall T waves |
| Para- myotonia Congenita | Hyper- kalemic Periodic Paralysis | Hypo- kalemic Periodic Paralysis | |
| Electrophysiological pattern | I | IV | V |
| Channel mutations | Sodium T1313M, R1448C | Sodium T704M | Calcium R528H |
| Short Exercise Test: | |||
| Post exercise myotonic potentials | Yes | No | No |
| CMAP amplitude change after First trial | Increase or decrease | Increase | No |
| CMAP amplitude change after second and third trial | Gradual increase | Gradual increase | No |
| Long Exercise Test: | |||
| Immediate change of CMAP amplitude | Decrease | Increase | No |
| Late change of CMAP amplitude | Decrease | Decrease | Decrease |
| Modified from Fournier et al, 2004.[9] | |||
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