The myopathy signs are a small set of bedside maneuvers that announce muscle disease. Each takes seconds to perform and identifies a pattern that is hard to mistake — the Gowers’ maneuver of climbing up the legs to stand, the percussion myotonia of the thenar eminence, the scapular winging of FSHD, the calf pseudohypertrophy of dystrophinopathy. The patient with muscle disease often presents with vague complaints — “I’m just weak,” “I can’t climb stairs,” “my arms feel heavy” — and the bedside exam translates these into a specific pattern that points to a category of disease. This page catalogs the major myopathy and myotonia signs and the diagnoses they suggest.

Gowers’ Maneuver

One of the most classical signs in pediatric neurology and a reliable indicator of proximal lower extremity weakness in adults as well. Ask the patient to rise from the floor without using their arms. The patient with significant proximal lower extremity weakness — typically from Duchenne muscular dystrophy in children, polymyositis or limb-girdle dystrophy in adults — uses the arms to “climb up the legs,” placing the hands on the thighs and walking them up toward the knees, then up to the hips, before achieving standing.

The maneuver is sensitive to subclinical proximal weakness and is positive even in mild myopathy. It is the single most useful bedside test in any child or adult with a possible myopathic complaint.

Percussion Myotonia

The bedside test for muscle myotonia. Tap the thenar eminence (or the deltoid, or the extensor digitorum) firmly with a reflex hammer. The normal muscle gives a brief contraction and immediately relaxes. The myotonic muscle produces a sustained contraction with delayed relaxation — a visible “dimple” or sustained muscle bunching that lingers for several seconds before slowly resolving.

Percussion myotonia is classical for myotonic dystrophy (especially type 1) and myotonia congenita. It can also be seen in paramyotonia congenita and the channelopathies. The duration of the contraction varies from a few seconds (mild) to many seconds (severe).

Grip Myotonia

The patient makes a tight fist and is asked to suddenly open the hand. The myotonic patient cannot release quickly — the fingers gradually unfold over several seconds. The test is particularly useful when percussion myotonia is subtle.

In myotonic dystrophy, grip myotonia often improves with warmth and worsens with cold. In paramyotonia congenita, the opposite is true — cold dramatically worsens the myotonia, and repetitive use worsens it further (paradoxical myotonia). These features can suggest the underlying channelopathy at the bedside.

Lid Myotonia

Have the patient close the eyes tightly and then open them suddenly. In myotonic dystrophy, the lids open slowly and incompletely, with a peculiar lid lag that may leave the upper sclera visible. Lid myotonia can be one of the earlier features in subtle disease.

The Myotonic Facial Appearance

Myotonic dystrophy type 1 produces a characteristic facial appearance over years — a long, narrow face, temporal wasting, masseter atrophy, frontal balding, and ptosis. The “myopathic facies” of inverted V-shaped mouth (from facial muscle weakness) and the long, lean facial structure are recognizable at a glance. Combined with grip myotonia and progressive distal weakness, the diagnosis is often made by appearance and bedside exam alone.

Scapular Winging Patterns

Scapular winging can have several patterns:

FSHD pattern: the scapula appears to “ride up” with shoulder abduction. The trapezius is preserved but the rhomboids and serratus anterior are weak, producing a characteristic forward-and-upward riding of the scapula that exposes the medial border.
Serratus anterior weakness (long thoracic nerve): the medial border of the scapula lifts off the chest wall when the patient pushes against a wall with the arms extended.
Trapezius weakness (CN XI): the scapula sits lower and laterally placed; winging on resisted shoulder abduction.

FSHD Pattern

Facioscapulohumeral muscular dystrophy is recognizable at a glance once you know the pattern. Features:

Facial weakness: difficulty closing the eyes tightly (orbicularis oculi weakness), inability to whistle or smile broadly. The patient may not be able to close the eyes during sleep.
Scapular winging with characteristic riding pattern.
Pectoralis major atrophy with preserved deltoid producing a “Popeye” appearance of the upper arms.
Foot drop from tibialis anterior weakness.
Asymmetric onset: typically more weakness on one side than the other.

The combination is highly characteristic and often allows clinical diagnosis before genetic testing.

Inclusion Body Myositis Pattern

Inclusion body myositis (IBM) has a peculiar pattern that distinguishes it from other inflammatory myopathies and from motor neuron disease:

Asymmetric, focal weakness: unlike the symmetric proximal pattern of polymyositis.
Finger flexor weakness: the patient cannot make a tight fist; finger flexor strength is dramatically reduced compared to wrist flexors and other forearm muscles. This is one of the most characteristic features.
Quadriceps weakness: difficulty climbing stairs and rising from chairs.
Foot drop in some cases.
Resistance to immunosuppression: unlike polymyositis and dermatomyositis, IBM responds poorly to steroids and immunomodulating drugs.
Older patients: typically over age 50.

The combination of asymmetric onset, prominent finger flexor weakness, quadriceps weakness, and resistance to treatment in an older patient is essentially diagnostic of IBM.

Dermatomyositis Patterns

Dermatomyositis combines proximal weakness with characteristic skin findings:

Heliotrope rash: violaceous discoloration of the upper eyelids, often with periorbital edema.
Gottron papules: erythematous, scaly plaques over the knuckles, elbows, and knees.
V-sign: erythematous rash on the anterior chest and neck.
Shawl sign: erythematous rash over the upper back and shoulders.
Mechanic’s hands: rough, hyperkeratotic skin on the radial side of the fingers (also seen in antisynthetase syndrome).
Periungual nailfold capillary changes: visible on close inspection or with capillaroscopy.

The combination of proximal weakness with these skin features is essentially diagnostic. Always look for malignancy in dermatomyositis (paraneoplastic in adults).

Pseudohypertrophy

Calf pseudohypertrophy in young boys: Duchenne muscular dystrophy. The calves appear bulky but are weak — muscle replaced by fat and fibrous tissue.
Calf pseudohypertrophy in adults: Becker muscular dystrophy, some limb-girdle dystrophies, rarely amyloid myopathy.
Tongue and deltoid pseudohypertrophy: described in some dystrophies but less common.

Trendelenburg Gait Pattern

Bilateral proximal lower extremity weakness — typical of myopathy — produces a side-to-side waddling gait. With each step, the contralateral hip drops because the weight-bearing leg’s hip abductors cannot stabilize the pelvis. The patient compensates by leaning the trunk over the weight-bearing leg, producing a rolling gait. Detailed in the Gait & Station page.

Tests for Specific Myopathies

Statin Myopathy

Statin-induced myopathy is the most common iatrogenic myopathy. Features:

Symmetric proximal weakness, often with myalgia.
Tenderness of affected muscles.
Elevated CK (often markedly).
Resolution within weeks of statin discontinuation in most cases.

A small subset of patients develop necrotizing autoimmune myopathy associated with anti-HMG-CoA reductase antibodies; this immune-mediated form does not resolve with statin discontinuation and requires immunosuppression.

Steroid Myopathy

Chronic corticosteroid use produces proximal myopathy with relatively normal CK. The classical history is a patient on chronic prednisone who has gradually become weak; rising from chairs and climbing stairs are early difficulties. CK is normal — unlike inflammatory or toxic myopathies — and the diagnosis is clinical and historical.

Thyroid Myopathy

Hypothyroid myopathy: proximal weakness, sometimes with myxedema; CK often elevated. Treatment is thyroid hormone replacement; weakness improves over weeks.
Hyperthyroid (thyrotoxic) myopathy: proximal weakness, often with substantial weight loss; CK normal or mildly elevated. Treatment is correction of thyroid status.

Periodic Paralysis

Channelopathies producing episodic weakness lasting hours to days. Hyperkalemic periodic paralysis (SCN4A mutations) often has associated myotonia and is triggered by potassium loading or rest after exercise. Hypokalemic periodic paralysis (CACNA1S or SCN4A mutations) is triggered by carbohydrate meals, stress, or exercise. Both can be inherited or rarely paraneoplastic.

Mitochondrial Myopathies

Often present with exercise intolerance and easy fatigability, sometimes with ophthalmoplegia, ptosis, or pigmentary retinopathy. The clinical clue is exercise intolerance disproportionate to the apparent weakness — the patient can produce reasonable strength briefly but fatigues quickly. Elevated lactate at rest or after exercise is suggestive.

Tests of Muscle Tenderness

Palpation of muscles can reveal tenderness in inflammatory myopathies (polymyositis, dermatomyositis), in toxic myopathies (statins), and in some metabolic disorders. Tenderness combined with weakness in a proximal distribution points to inflammatory or toxic causes; weakness without tenderness is more typical of muscular dystrophy.

Cardiac and Respiratory Involvement

Many myopathies have cardiac and respiratory complications that must be considered:

Duchenne and Becker muscular dystrophies: cardiomyopathy is common and may be the cause of death. Cardiac screening is essential.
Myotonic dystrophy: cardiac conduction abnormalities (heart block, atrial arrhythmias) are common. ECG screening at diagnosis and periodically.
Inflammatory myopathies: cardiac involvement (myocarditis) and interstitial lung disease can occur.
Limb-girdle dystrophies: some subtypes have cardiac and respiratory involvement.
Mitochondrial myopathies: cardiac involvement (especially Kearns-Sayre syndrome with cardiac conduction defects).

Always ask about exercise tolerance, dyspnea, palpitations, and syncope in any patient with myopathy.

🔍 Did You Know?

The pattern of weakness in inclusion body myositis is so peculiar that it allows clinical diagnosis even before muscle biopsy. The combination of finger flexor weakness (the patient cannot make a tight fist) with quadriceps weakness (difficulty rising from chairs and climbing stairs), often asymmetric, in a patient over age 50, with relative sparing of other muscle groups, is essentially pathognomonic. The condition is the most common inflammatory myopathy in older adults and is sometimes initially misdiagnosed as motor neuron disease because of the focal pattern. The key distinction is the lack of upper motor neuron signs, the absence of fasciculations and bulbar involvement (in IBM), and the typically slow progression over years.

Pitfalls and Pearls

Gowers’ maneuver is the single most useful bedside test for proximal lower extremity weakness. Ask any patient with possible myopathy to rise from the floor without using their arms.
Percussion myotonia is highly specific. Tap the thenar eminence; sustained dimple confirms myotonia.
Grip myotonia worsens with cold in paramyotonia congenita; improves with warm-up in myotonic dystrophy. The history of cold sensitivity is a clue.
The myopathic facial appearance of myotonic dystrophy — long, narrow face, temporal wasting, ptosis, frontal balding — is often recognizable at a glance.
FSHD has a characteristic scapular pattern with the scapula riding up and the medial border exposed. Combined with facial weakness and pectoralis atrophy, the diagnosis is often clinical.
Inclusion body myositis presents with finger flexor weakness and quadriceps weakness in older patients. Asymmetric, focal, treatment-resistant.
Dermatomyositis skin findings are essentially diagnostic when combined with proximal weakness. Always look for them.
Calf pseudohypertrophy in a young boy is Duchenne until proven otherwise. Send a CK and obtain genetic testing.
Always check medication list in any unexplained proximal weakness. Statins are the most common culprits.
Always check thyroid function and CK in unexplained proximal weakness.
Trendelenburg (waddling) gait in a patient with proximal weakness points to myopathy.
Many myopathies have cardiac and respiratory complications. Screen for these at diagnosis and follow them over time.
Painful inflammatory myopathy in an adult warrants malignancy screening, particularly for dermatomyositis.

References

Campbell WW. DeJong’s The Neurologic Examination. 7th ed. Philadelphia: Lippincott Williams & Wilkins; 2013. Chapter 27.
Amato AA, Russell JA. Neuromuscular Disorders. 2nd ed. McGraw-Hill; 2016.
Dimachkie MM, Barohn RJ. Inclusion body myositis. Neurol Clin. 2014;32(3):629-646.
Tawil R, Van Der Maarel SM. Facioscapulohumeral muscular dystrophy. Muscle Nerve. 2006;34(1):1-15.
Lundberg IE, Tjärnlund A, Bottai M, et al. 2017 EULAR/ACR classification criteria for adult and juvenile idiopathic inflammatory myopathies. Ann Rheum Dis. 2017;76(12):1955-1964.
Statland JM, Tawil R. Myotonic dystrophy. Continuum (Minneap Minn). 2014;20(6):1668-1681.
Bushby K, Finkel R, Birnkrant DJ, et al. Diagnosis and management of Duchenne muscular dystrophy. Lancet Neurol. 2010;9(1):77-93.