NEUROMYTHOLOGY

Pyramidal weakness
Charles Mark Wiles
Department of Neurology, Guys & The signs associated with an upper motor by comparison with the normal side (if
St Thomas’ NHS Foundation neurone syndrome vary considerably weakness is unilateral) or from our
Trust, London, UK
between text books. Often included are knowledge of normal strength in
Correspondence to loss of dexterous, rapid and fractionated different muscle groups acquired by
Professor Charles Mark Wiles, voluntary movements, variation of move- examination of many patients. Whether
Department of Neurology, Guy’s
and St. Thomas’ NHS Foundation
ment with mode of activation (prime or not we can ‘break’ a contraction
Trust, 3rd Floor, Lambeth Wing, mover, synergist, antagonist), weakness, depends on the muscle group, the indi-
St. Thomas’ Hospital, London SE1 various postural signs (eg, pronator drift), vidual patient and our own strength but
7EH, UK; [email protected] time-dependent tonal and tendon reflex does not, per se, indicate weakness. I
Accepted 05 January 2017
changes and the extensor plantar know that I can sometimes break
response. Russell Brain’s first edition of maximum voluntary elbow extension but
Diseases of the Nervous System (1933, not flexion in a healthy subject, rarely hip
pp 6-8) refers to movements of flexion or knee extension, and virtually never
being stronger than extension in the ankle plantar flexion. Only the most
upper limb and extension movements determined examiner can generate a
stronger than flexion in the lower limb as manual counterforce of 200–300 N or
one negative feature of a unilateral pyra- more. Thus, we expand clinical testing to
midal (internal capsular) lesion, albeit use the subject’s body weight to stand
attributing this ‘probably’ to the distribu- from sitting (hip extension) or to hop
tion of hypertonia. However, this pattern (ankle plantar flexion). The quality of the
of ‘pyramidal weakness’ (also including patient’s effort, for example, ‘give way’
shoulder abduction) has since been weakness, the rate of force build up, early
emphasised in many text books as being fatigue or disparity between ‘make’ and
characteristic of an upper motor neurone ‘break’ forces may also inform diagnosis.
lesion. In healthy men and women aged 20–79
Early in my consultant career, I saw a years, quantified measurements of
man with acute pain in the low and mid isometric strength in positions similar to
back and weakness in both legs. He could those used in clinical examination show
barely stand, tone was normal and he had that strength of shoulder abduction is
weakness of hip flexion more than exten- about 0.6–0.8 that of adduction; elbow
sion, knee flexion more than extension extension about 0.7–0.9 that of flexion;
and ankle dorsiflexion much more than and wrist extension about 0.4–0.6 that of
plantar flexion: tendon reflexes were flexion. Similar ratios pertain for hip and
brisk, and plantar responses silent with knee flexors compared with extensors,
joint position and vibration sense loss in and ankle dorsiflexion is 0.3–0.4 that of
the toes and distal cutaneous loss. I diag- plantar flexion.1 Thus, a ‘super-strong’
nosed an evolving spinal cord lesion. examiner testing a healthy individual
However within hours he had obvious would find a so-called ’pyramidal
facial, bulbar and arm weakness (also in a pattern’. If strength were reduced by
‘pyramidal pattern’) and absent tendon 30% in both flexors and extensors (ie, no
reflexes: he turned out to have Guillain- selective weakness), more examiners
Barre syndrome. I was dismayed by being would detect a ‘pyramidal pattern’.
initially misled about localisation mainly Whether lesions engaging the cortico-
through the distribution of weakness. spinal pathways cause a selective
When manually examining strength in ‘pyramidal pattern’ of weakness was
specific muscle groups (figure 1), we directly addressed in both upper and
usually encourage the patient to build lower limbs using fixed dynamometry,
up their strongest contraction in an with allowances for gravitational
To cite: Wiles CM. Pract approximately isometric manner. We then torques. 2–4
Physiological flexors and
Neurol 2017;0:1–2.
judge it to be normal or otherwise either extensors were equally affected in the

Wiles CM. Pract Neurol 2017;0:1–2. doi:10.1136/practneurol-2016-001584 1

NEUROMYTHOLOGY

‘Pyramidal weakness’ may be an illusion resulting

from manual testing, the natural strength of muscle
groups and the distributed nature of (sometimes
marked) increased tone in an upper motor neurone
lesion. Undergraduates, neurology trainees and even
experienced clinicians should probably not make too
much of it. A deeper analysis of clinical features of
the upper motor neurone syndrome caused by lesions
at differing levels in the corticobulbar/corticospinal
pathways using modern imaging and quantification of
signs would be worthwhile.

Figure 1 The examiner’s counterforce (arrows) required to Competing interests None declared.
’break’ a maximum voluntary contraction of elbow flexion (red) Provenance and peer review Commissioned; externally peer
or extension (blue) in a healthy male subject. reviewed. This paper was reviewed by Martin Turner, Oxford, UK.

lower limbs of both hemiparetic and paraparetic

REFERENCES
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2 Wiles CM. Pract Neurol 2017;0:1–2. doi:10.1136/practneurol-2016-001584