Velenberg Zakanchenko (alternating syndrome

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alternating-hemiplegia-abnormal-cerebralMedial medullary syndrome, also known as inferior alternating syndrome, hyploglossal alternating hemiplegia, or lower alternating hemiplegia, is a set of clinical features resulting from occlusion of vertebral artery or of branch of vertebral or lower basilar artery. This results in the infaction of medial part of the medulla oblongata.

It is also known as "Dejerine syndrome".

The infarction leads to death of the ipsilateral medullary pyramid, the ipsilateral medial leminiscus, and hypoglossal nerve fibers that pass through the medulla.

The spinothalamic tract is spared because it is located more laterally in the brainstem and is not supplied by the anterior spinal artery, but rather by the vertebral and posterior inferior cerebellar arteries. The trigeminal nucleus is also spared, since most of it is higher up in the pons, and the spinal part of it found in the medulla is lateral to the infarct.

The condition usually consists of:

Description

Source of damage

 

a deviation of the tongue to the ipsilateral side of the infarct on attempted protrusion, caused by muscle weakness on the ipsilateral side

hypoglossal nerve fibers

 

limb weakness (or hemiplegia, depending on severity), on the contralateral side of the infarct

medullary pyramid and hence to the corticospinal fibers of the pyramidal tract

 

a loss of discriminative touch, conscious proprioception, and vibration sense on the contralateral side of the infarct

medial leminiscus

 

Sensation to the face is preserved, due to the sparing of the trigeminal nucleus.

The syndrome is said to be "alternating" because the lesion causes symptoms both contralaterally and ipsilaterally. Sensation of pain and temperature is preserved, because the spinothalamic tract is located more laterally in the brainstem and is also not supplied by the anterior spinal artery (instead supplied by the posterior inferior cerebellar arteries and the vertebral arteries).

Brown-Séquard syndrome

Brown-Séquard syndrome, also known as Brown-Séquard's hemiplegia and Brown-Séquard's paralysis, is a loss of sensation and motor function (paralysis and ataxia) that is caused by the lateral hemisection (cutting) of the spinal cord. Other synonyms are crossed hemiplegia, hemiparaplegic syndrome, hemiplegia et hemiparaplegia spinalis and spinal hemiparaplegi

Any presentation of spinal injury that is an incomplete lesion can be called a partial Brown-Séquard or incomplete Brown-Séquard syndrome, so long as it has characterized by features of a motor loss and numbness to touch and vibration on the same side of the spinal injury and loss of pain and temperature sensation on the opposite side.

Brown-Séquard syndrome may be caused by a spinal cord tumor, trauma (such as a gunshot wound or puncture wound to the neck or back), ischemia (obstruction of a blood vessel), or infectious or inflammatory diseases such as tuberculosis, or multiple sclerosis.

Brown-Séquard syndrome is an incomplete spinal cord lesion characterized by clinical presentation reflecting hemisection of the spinal cord (cutting the spinal cord in half on one or the other side). It is diagnosed by finding motor (muscle) paralysis on the same side as the lesion and deficits in pain and temperature sensation on the opposite side on physical exam. This is called ipsilateral (on the same side as the spinal cord lesion) hemiplegia and contralateral (on the opposite side) pain and temperature sensation deficits. The loss of sensation on the opposite side of the lesion is because these nerve fibers of the spinothalamic tract cross the spinal cord. In its pure form, it is rarely seen. Incomplete forms are also observed. The most common cause is penetrating trauma such as a gunshot wound or stab wound to the spinal cord. This may be seen most often in the cervical (neck) or thoracic spine. Other causes are tumors, bleeding episodes, tuberculosis, and multiple sclerosis.

The presentation can be progressive and incomplete. It can advance from a typical Brown-Séquard syndrome to complete paralysis. It is not always permanent, and progression or resolution depends on the severity of the original spinal cord injury and the underlying pathology that caused it in the first place.

Romberg's test

Romberg's test is a neurological test that is used to assess the dorsal columns of the spinal cord,[1] which are essential for joint position sense (proprioception) and vibration sense.

A positive Romberg test suggests that ataxia is sensory in nature, i.e. depending on loss of proprioception. A negative Romberg test suggests that ataxia is cerebellar in nature, i.e. depending on localized cerebellar dysfunction instead.

 

Ask the subject to stand erect with feet together and eyes closed. Stand close by as a precaution in order to stop the person from falling over and hurting himself. Watch the movement of the body in relation to a perpendicular object behind the subject (corner of the room, door, window etc). A positive sign is noted when a swaying, sometimes irregular swaying and even toppling over occurs. The essential feature is that the patient becomes more unsteady with eyes closed.

The essential features of the test are as follows:

  1. the subject stands with feet together, eyes open and hands by the sides.
  2. the subject closes the eyes while the examiner observes for a full minute.

Because the examiner is trying to elicit whether the patient falls when the eyes are closed, it is advisable to stand ready to catch the falling patient. For large subjects, a strong assistant is recommended.

Romberg's test is positive if the patient sways or falls while the patient's eyes are closed.

Patients with a positive result are said to demonstrate Romberg's sign or Rombergism. They can also be described as Romberg's positive. The basis of this test is that balance comes from the combination of several neurological systems, namely proprioception, vestibular input, and vision. If any two of these systems are working the person should be able to demonstrate a fair degree of balance. The key to the test is that vision is taken away by asking the patient to close their eyes. This leaves only two of the three systems remaining and if there is a vestibular disorder (cerebellar dysfunction) or a sensory disorder (proprioceptive dysfunction) the patient will become much more imbalanced.

Maintaining balance while standing in the stationary position relies on intact sensory pathways, sensorimotor integration centers and motor pathways.

The main sensory inputs are:

  1. Joint position sense (proprioception), carried in the dorsal columns of the spinal cord;
  2. Vision
  3. Vestibular apparatus

Crucially, the brain can obtain sufficient information to maintain balance if any two of the three systems are intact.

Sensorimotor integration is carried out by the cerebellum and by the dorsal column-medial lemniscus tract. The motor pathway is the corticospinal (pyramidal) tract and the medial and lateral vestibular tracts.

The first stage of the test (standing with the eyes open), demonstrates that at least two of the three sensory pathways is intact, and that sensorimotor integration and the motor pathway are functioning.

In the second stage, the visual pathway is removed by closing the eyes, known as a "sharpened Romberg". If the proprioceptive and vestibular pathways are intact, balance will be maintained. But if proprioception is defective, two of the sensory inputs will be absent and the patient will sway then fall.

The sharpened Romberg does have an early learning effect that will plateau between the third and fourth attempts.

+ve Romberg

Romberg's test is positive in conditions causing sensory ataxia such as:

  • Conditions affecting the dorsal columns of the spinal cord, such as tabes dorsalis (neurosyphilis), in which it was first described.
  • Conditions affecting the sensory nerves (sensory peripheral neuropathies), such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).
  • Friedreich's Ataxia

Meningeal Syndrome (Meningism)

Meningism is the triad of nuchal rigidity (neck stiffness), photophobia (intolerance of bright light) and headache. It is a sign of irritation of the meninges, such as seen in meningitis, subarachnoid hemorrhages and various other diseases. "Meningismus" is the term used when the above listed symptoms are present without actual infection or inflammation; usually it is seen in concordance with other acute illnesses in the pediatric population. Related clinical signs include Kernig's sign and three signs all named Brudzinski's sign.

The main clinical signs that indicate meningism are nuchal rigidity, Kernig's sign and Brudzinski's signs. None of the signs are particularly sensitive; in adults with meningitis, nuchal rigidity was present in 30% and Kernig's or Brudzinski's sign only in 5%.

Nuchal rigidity

Nuchal rigidity is the inability to flex the head forward due to rigidity of the neck muscles; if flexion of the neck is painful but full range of motion is present, nuchal rigidity is absent.

Kernig's sign

Kernig's sign (after Waldemar Kernig (1840-1917), a Baltic German neurologist) is positive when the leg is fully bent in the hip and knee, and subsequent extension in the knee is painful (leading to resistance).[3]. This may indicate subarachnoid haemorrhage or meningitis. Patients may also show opisthotonus—spasm of the whole body that leads to legs and head being bent back and body bowed backwards.

Brudzinski's signs

Jozef Brodzinski (1874-1917), a Polish pediatrician, is credited with several signs in meningitis. The most commonly used sign (Brudzinski's neck sign) is the appearance of involuntary lifting of the legs in meningeal irritation when lifting a patient's head.

Other signs attributed to Brudzinski:

  • The symphyseal sign, in which pressure on the pubic symphysis leads to abduction of the leg and reflexive hip and knee flexion.
  • The cheek sign, in which pressure on the cheek below the zygoma leads to rising and flexion in the forearm.
  • Brudzinski's reflex, in which passive flexion of one knee into the abdomen leads to involuntary flexion in the opposite leg, and stretching of a limb that was flexed leads to contralateral extension.

 

Last modified onMonday, 06 January 2014 17:32
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