Upper motor neuron syndrome (UMNS) is the

motor control Motor control is the regulation of movement in organisms that possess a nervous system. Motor control includes reflexes as well as directed movement. To control movement, the nervous system must integrate multimodal sensory information (both f ...

changes that can occur in

skeletal muscle Skeletal muscles (commonly referred to as muscles) are organs of the vertebrate muscular system and typically are attached by tendons to bones of a skeleton. The muscle cells of skeletal muscles are much longer than in the other types of m ...

after an

upper motor neuron lesion An upper motor neuron lesion (also known as pyramidal insufficiency) Is an injury or abnormality that occurs in the neural pathway above the anterior horn cell of the spinal cord or motor nuclei of the cranial nerves. Conversely, a lower motor ...

. Following upper motor neuron lesions, affected muscles potentially have many features of altered performance including: *

weakness Weakness is a symptom of a number of different conditions. The causes are many and can be divided into conditions that have true or perceived muscle weakness. True muscle weakness is a primary symptom of a variety of skeletal muscle diseases, i ...

(decreased ability for the muscle to generate force) *decreased

including decreased speed, accuracy and

dexterity Fine motor skill (or dexterity) is the coordination of small muscles in movement with the eyes, hands and fingers. The complex levels of manual dexterity that humans exhibit can be related to the nervous system. Fine motor skills aid in the grow ...

*altered

muscle tone In physiology, medicine, and anatomy, muscle tone (residual muscle tension or tonus) is the continuous and passive partial contraction of the muscles, or the muscle's resistance to passive stretch during resting state.O’Sullivan, S. B. (2007). ...

(hypotonia or hypertonia) – a decrease or increase in the baseline level of muscle activity *decreased endurance *exaggerated deep tendon reflexes including

spasticity Spasticity () is a feature of altered skeletal muscle performance with a combination of paralysis, increased tendon reflex activity, and hypertonia. It is also colloquially referred to as an unusual "tightness", stiffness, or "pull" of muscles ...

, and

clonus Clonus is a set of involuntary and rhythmic muscular contractions and relaxations. Clonus is a sign of certain neurological conditions, particularly associated with upper motor neuron lesions involving descending motor pathways, and in many cas ...

(a series of involuntary rapid muscle contractions) Such signs are collectively termed the "upper motor neuron syndrome". Affected muscles typically show multiple signs, with severity depending on the degree of damage and other factors that influence

. In neuroanatomical circles, it is often joked, for example, that hemisection of the cervical spinal cord leads to an "upper lower motor neuron syndrome and a lower upper motor neuron syndrome". The saying refers to lower motor neuron symptoms in the upper extremity (arm) and upper motor neurons symptoms in the lower extremity (leg). Health professionals' understanding of impairments in muscles after an

has progressed considerably in recent decades. However, a diagnosis of "spasticity" is still often used interchangeably with upper motor neuron syndrome, and it is not unusual to see patients labeled as spastic who demonstrate an array of UMN findings.Ivanhoe CB, Reistetter TA: Spasticity: The misunderstood part of the upper motor neuron syndrome. Am J Phys Med Rehabil 2004;83(suppl):S3–S9.

Spasticity Spasticity () is a feature of altered skeletal muscle performance with a combination of paralysis, increased tendon reflex activity, and hypertonia. It is also colloquially referred to as an unusual "tightness", stiffness, or "pull" of muscles ...

is an exaggerated

stretch reflex The stretch reflex (myotatic reflex), or more accurately "muscle stretch reflex", is a muscle contraction in response to stretching within the muscle. The reflex functions to maintain the muscle at a constant length. The term deep tendon reflex is ...

, which means that a muscle has a reflex contraction when stretched, and that this contraction is stronger when the stretch is applied more quickly. The commonly quoted definition by Lance (1980) describes "a motor disorder, characterised by a velocity-dependent increase in tonic stretch reflexes with exaggerated tendon jerks, resulting from hyper-excitability of the stretch reflex as one component of the upper motor neurone (UMN) syndrome". Spasticity is a common feature of muscle performance after upper motor neuron lesions, but is generally of much less clinical significance than other features such as decreased strength, decreased control and decreased

endurance Endurance (also related to sufferance, resilience, constitution, fortitude, and hardiness) is the ability of an organism to exert itself and remain active for a long period of time, as well as its ability to resist, withstand, recover from an ...

. The confusion in the use of the terminology complicates assessment and treatment planning by health professionals, as many confuse the other findings of upper motor neuron syndrome and describe them as spasticity. This confusion potentially leaves health professionals attempting to inhibit an exaggerated stretch reflex to improve muscle performance, potentially leaving more significant UMNS changes such as weakness unaddressed. Improved understanding of the multiple features of the upper motor neuron syndrome supports more rigorous assessment, and improved treatment planning.

Presentation

The upper motor neuron syndrome signs are seen in conditions where motor areas in the

brain The brain is an organ that serves as the center of the nervous system in all vertebrate and most invertebrate animals. It consists of nervous tissue and is typically located in the head ( cephalization), usually near organs for special ...

and/or

spinal cord The spinal cord is a long, thin, tubular structure made up of nervous tissue, which extends from the medulla oblongata in the brainstem to the lumbar region of the vertebral column (backbone). The backbone encloses the central canal of the spin ...

are damaged or fail to develop normally. These include

spinal cord injury A spinal cord injury (SCI) is damage to the spinal cord that causes temporary or permanent changes in its function. Symptoms may include loss of muscle function, sensation, or autonomic function in the parts of the body served by the spinal co ...

cerebral palsy Cerebral palsy (CP) is a group of movement disorders that appear in early childhood. Signs and symptoms vary among people and over time, but include poor coordination, spasticity, stiff muscles, Paresis, weak muscles, and tremors. There may be p ...

, multiple sclerosis and

acquired brain injury Acquired brain injury (ABI) is brain damage caused by events after birth, rather than as part of a genetic or congenital disorder such as fetal alcohol syndrome, perinatal illness or perinatal hypoxia. ABI can result in cognitive, physical, em ...

including stroke. The impact of impairment of muscles for an individual is problems with movement, and posture, which often affects their function.

Diagnosis

Assessment of motor control may involve several health professionals depending on the affected individual's situation, and the severity of their condition. This may include

physical therapists Physical therapy (PT), also known as physiotherapy, is one of the allied health professions. It is provided by physical therapists who promote, maintain, or restore health through physical examination, diagnosis, management, prognosis, patien ...

, physicians (including

neurologists Neurology (from el, νεῦρον (neûron), "string, nerve" and the suffix -logia, "study of") is the branch of medicine dealing with the diagnosis and treatment of all categories of conditions and disease involving the brain, the spinal c ...

and

psychiatrists A psychiatrist is a physician who specializes in psychiatry, the branch of medicine devoted to the diagnosis, prevention, study, and treatment of mental disorders. Psychiatrists are physicians and evaluate patients to determine whether their ...

) and rehabilitation physicians,

orthotists An orthotist is a healthcare professional who specializes in the provision of orthoses. An orthotist has an overall responsibly of orthotics treatment, who can supervise and mentor the practice of other personnel. They are clinicians trained to ...

occupational therapists Occupational therapists (OTs) are health care professionals specializing in occupational therapy and occupational science. OTs and occupational therapy assistants (OTAs) use scientific bases and a holistic perspective to promote a person's abilit ...

, and speech-language pathologists. Assessment is needed of the affected individual's goals, their function, and any symptoms that may be related to the movement disorder, such as pain. A thorough assessment then uses a clinical reasoning approach to determine why difficulties are occurring. Elements of assessment will include analysis of posture, active movement, muscle strength, movement control and coordination, and endurance, as well as muscle tone and spasticity. Impaired muscles typically demonstrate a loss of selective movement, including a loss of eccentric control (decreased ability to actively lengthen); this decreased active lengthening of a muscle is a key factor that limits motor control. While multiple muscles in a limb are usually affected in the Upper Motor Neuron Syndrome, there is usually an imbalance of muscle activity (muscle tone), such that there is a stronger pull on one side of a joint, such as into elbow flexion. Decreasing the degree of this imbalance is a common focus of muscle strengthening programs. Impaired motor control also typically features a loss of stabilisation of an affected limb or the head from the trunk, so a thorough assessment requires this to be analysed as well, and exercise to improve

proximal Standard anatomical terms of location are used to unambiguously describe the anatomy of animals, including humans. The terms, typically derived from Latin or Greek roots, describe something in its standard anatomical position. This position ...

stability may be indicated. Secondary effects are likely to impact on assessment of impaired muscles. If muscle tone is assessed with passive muscle lengthening, increased muscle

stiffness Stiffness is the extent to which an object resists deformation in response to an applied force. The complementary concept is flexibility or pliability: the more flexible an object is, the less stiff it is. Calculations The stiffness, k, of a b ...

may affect the feeling of resistance to passive stretch, in addition to neurological resistance to stretch. Other secondary changes such as loss of muscle fibres following acquired muscle weakness are likely to compound the weakness arising from the upper motor neuron lesion. In severely affected muscles, there may be marked secondary changes, such as muscle

contracture In pathology, a contracture is a permanent shortening of a muscle or joint. It is usually in response to prolonged hypertonic spasticity in a concentrated muscle area, such as is seen in the tightest muscles of people with conditions like spasti ...

, particularly if management has been delayed or absent.

Treatment

Treatment should be based on assessment by the relevant health professionals. For muscles with mild-to-moderate impairment, exercise should be the mainstay of management, and is likely to need to be prescribed by a

physical therapist Physical therapy (PT), also known as physiotherapy, is one of the allied health professions. It is provided by physical therapists who promote, maintain, or restore health through physical examination, diagnosis, management, prognosis, pat ...

or other health professional skilled in neurological rehabilitation. Muscles with severe impairment are likely to be more limited in their ability to exercise, and may require help to do this. They may require additional interventions, to manage the greater neurological impairment and also greater secondary complications. These interventions may include serial casting, flexibility exercise such as sustained positioning programs, and medical interventions. Research has clearly shown that exercise is beneficial for impaired muscles, even though it was previously believed that strength exercise would ''increase'' muscle tone and impair muscle performance further. Also, in previous decades there has been a strong focus on other interventions for impaired muscles, particularly stretching and splinting, but the evidence does not support these as effective. One of the challenges for health professionals working with UMNS movement disorders is that the degree of muscle weakness makes developing an exercise programme difficult. For muscles that lack any volitional control, such as after complete spinal cord injury, exercise may be assisted, and may require equipment, such as using a standing frame to sustain a standing position. Often, muscles require specific stimulation to achieve small amounts of activity, which is most often achieved by weight-bearing (e.g. positioning and supporting a limb such that it supports body weight) or by stimulation to the muscle belly (such as electrical stimulation or vibration). Medical interventions may include such medications as

baclofen Baclofen, sold under the brand name Lioresal among others, is a medication used to treat muscle spasticity such as from a spinal cord injury or multiple sclerosis. It may also be used for hiccups and muscle spasms near the end of life. It is ...

diazepam Diazepam, first marketed as Valium, is a medicine of the benzodiazepine family that acts as an anxiolytic. It is commonly used to treat a range of conditions, including anxiety, seizures, alcohol withdrawal syndrome, muscle spasms, insomnia, ...

dantrolene Dantrolene sodium, sold under the brand name Dantrium among others, is a postsynaptic muscle relaxant that lessens excitation-contraction coupling in muscle cells. It achieves this by inhibiting Ca2+ ions release from sarcoplasmic reticulum ...

, or

clonazepam Clonazepam, sold under the brand names Klonopin and Rivotril, is a medication used to prevent and treat seizures, panic disorder, anxiety disorders, and the movement disorder known as akathisia. It is a tranquilizer of the benzodiazepine class ...

Phenol Phenol (also called carbolic acid) is an aromatic organic compound with the molecular formula . It is a white crystalline solid that is volatile. The molecule consists of a phenyl group () bonded to a hydroxy group (). Mildly acidic, it ...

injections or

botulinum toxin Botulinum toxin, or botulinum neurotoxin (BoNT), is a neurotoxic protein produced by the bacterium ''Clostridium botulinum'' and related species. It prevents the release of the neurotransmitter acetylcholine from axon endings at the neuromu ...

injections into a muscle belly of the upper or lower extremities can be used to attempt to dampen the signals between nerve and muscle. The effectiveness of medications varies between individuals, and varies based on location of the upper motor neuron lesion (in the brain or the spinal cord). Medications are commonly used for movement disorders, but research has not shown functional benefit for some drugs.Taricco M, Adone R, Pagliacci C, Telaro E. Pharmacological interventions for spasticity following spinal cord injury. Cochrane Database of Systematic Reviews 2000, Issue 2. Art. No.: CD001131. Some studies have shown that medications have been effective in decreasing spasticity, but that this has not been accompanied by functional benefits.

References

{{Reflist Motor control Syndromes

Presentation

Diagnosis

Treatment

See also

References