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potential exists for considerable interexaminer variability It has been documented that patients may lose 80% or more of their motor units in a given muscle before they receive a 3 or less MRC rating5 In the opinion of the author , it is a poor tool to measure motor de cits in UMN disease Increasingly in clinical trials, and to some extent in clinical practice, tools such as hand-held dynamometry are used in an attempt to measure strength in a more objective, linear, and reproducible manner The presence of scapular winging is an important and easily overlooked diagnostic clue in the assessment of neuromuscular disease The patients will be unable to raise their hand over their head effectively On observation, these patients may appear to have overly developed trapezius muscles (when viewed from the front) What is actually being seen is the superior margin of the scapula, which is elevated due to weakness of the scapular support muscles Other observations may include a crease in the anterior axillary fold and an abnormal hand position where the dorsum of the hand rather than the thumb faces forward, producing a simian arm position while standing These are both the result of abnormal positioning of the scapula with rotation of the entire shoulder joint anteriorly Scapular winging may be evident by simply looking at the patient from the rear It may be accentuated by a number of maneuvers depending on the pattern of affected muscles that are weak Scapular winging due to weakness of the serratus anterior causes the inferior tip of the scapula to be preferentially elevated off the ribcage and is deviated medially toward the spine It can be accentuated by having the patient push against a wall or slowly ex the arm at the shoulder anteriorly against resistance With scapular winging resulting from trapezius weakness, the entire medial border is elevated and is accentuated by attempted abduction of the arm at the shoulder against resistance The dynamics of scapular winging resulting from the more diffuse myopathic and motor neuron disorders are more complex Testing cranial nerve function in suspected neuromuscular disease is in part observational Eyelid position can offer signi cant insight The upper lid normally covers the upper limbus by a few millimeters or less, whereas the lower lid typically intersects the lower limbus This relationship might change with diseases that produce either ex- or endophthalmos or symmetrically on the basis of age-related loss of tissue tone Alteration of lid position may also be of a neuromuscular consequence For example, a sagging (ptotic) upper lid combined by a sagging lower lid exposing sclera below the lower limbus suggests concomitant weakness of muscles that both open and close the eye lids As these muscles have different innervations, this observation by itself may be a clue that the diagnosis is one capable of causing multifocal weakness with a predilection for cranial muscles, eg, myasthenia gravis Another observation of importance is the position of the eyebrow and the state of.

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frontalis contraction With ptosis, the eyebrow should be elevated and the ipsilateral frontalis contracted (if possible) in an attempt to compensate for the drooping lid If the patient is squinting, which may be confused with ptosis, the eyebrow descends and the frontalis is not activated The pupils should be examined, preferably, at least initially, in a dimly lit room to assess for the possibility of Horner s syndrome The lack of pupillary reactivity may represent an autonomic component of the patient s disorder Perhaps, the greatest value of the pupil examination in neuromuscular disease is to distinguish neuromuscular disorders causing ophthalmoparesis that spare the pupil from those that do not Myasthenia, diabetic third nerve palsies, and myopathic causes of ophthalmoparesis t into the former category Ophthalmoparesis with pupillary involvement may occur as a consequence of Guillain Barr syndrome and its variants and also due e to presynaptic disorders of neuromuscular transmission such as botulism Jaw weakness is uncommonly detected Weakness of jaw closing can be tested by having the patient close his or her mouth while the examiner attempts to force it open with the thumbs The index and middle ngers are placed on the side of the face and the fourth and fth digits on the side of the neck, in an attempt to stabilize the neck Testing the strength of jaw opening needs to be done cautiously, as both the patient s teeth and tongue may be injured if patient s effort or resistance is diminished Typically, the examiner places one hand on the back of the head to stabilize while force is delivered with the palm of the other hand on the under surface of the chin The examiner must be sensitive to any give on the part of the patient so as to stop pushing before the jaw potentially snaps shut in order to avert injury Facial strength can be assessed as previously mentioned by observation, either by a drooping lower lid or by patients inability to bury their eyelashes when asked to close their eyes forcibly The latter can be corroborated in a more objective fashion by using the index nger and thumb in an attempt to pry the eyelids open while the patient is rendering full effort Patient effort can be assessed by the presence or absence of Bell s phenomenon (upward deviation of the globe) if the eyelids can be effectively separated Tongue strength is typically tested by having patients thrust their tongue in either cheek, ie, pocketing or against a tongue blade The examiner attempts to push it back to the midline by applying pressure to the tongue Sternocleidomastoid strength can be tested by rotating the head and applying isometric pressure to the chin To test neck exion, the patient is placed in either supine or sitting position, depending on the strength of the patient and the examiner, and asked to place the chin on the chest while the examiner pushes down on.

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