Brown-Séquard syndrome (BSS) is a type of incomplete spinal cord lesion with a particular clinical pattern, which is characterized by the paralysis and loss of proprioception on the body side of the injury (ipsilateral) and loss of pain and temperature sensation on the opposite one.
Presentation
The onset of the clinical manifestations is generally acute and progressive in nature. As previously said, complete hemisection is very rare. Incomplete hemisection, instead, is much more common and implies the appearance of signs and symptoms of other forms of spinal cord injury.
Pure BSS is generally characterized by the interruption of the lateral corticospinal tracts, the interruption of the posterior white column, and that of lateral spinothalamic tracts. Partial forms instead, are usually characterized by asymmetrical paresis with marked hypalgesia on the less paretic side.
At the level of the lesion it is possible to observe a total ipsilateral loss of position, light touch and vibration sensation. As previously said, controlateral loss of pain and temperature can be seen a few segments below the lesion, due to the particular architecture of spinothalamic tracts in the spinal cord. This is usually associated with ipsilateral spastic paraparesis with the loss of vibration and joint-position sense due to the damage of ipsilateral dorsal column fibres underneath the lesion.
The signs of the incomplete forms of BSS are usually the result of vascular impairment due to spinal cord compression or inflammatory lesions such as multiple sclerosis. Other conditions and syndromes which show similar clinical signs to those of BSS include spinal cord compression, stroke, multiple sclerosis, and spinal tumors.
Entire Body System
- Unable to Stand
Initially she was unable to stand without maximum assistance. MR imaging revealed transection of the left hemicord at the C5 level without cord hemorrhage. CTA of the neck was negative for vascular injury. [ncbi.nlm.nih.gov]
Respiratoric
- Sputum
Laboratory investigations in case of infectious etiologies like purified protein derivative and sputum for acid-fast bacilli along with chest x-ray. MRI or X-ray when traumatic injuries are in suspected as well as neoplastic causes. [statpearls.com]
- Pleural Effusion
Additional pulmonary complications include pneumonia, pulmonary embolism, pleural effusion, pneumothorax, and mucus plug. Gastrointestinal Complications include ileus, constipation, pancreatitis, abdominal rigidity, and stress ulceration. [neuropedia.net]
Musculoskeletal
- Hand Muscle Weakness
An 80-year-old woman with type 2 diabetes was admitted due to right-handed muscle weakness. [ncbi.nlm.nih.gov]
Neurologic
- Hemianesthesia
Organizations Publications Definition Brown-Sequard syndrome (BSS) is a rare neurological condition characterized by a lesion in the spinal cord which results in weakness or paralysis (hemiparaplegia) on one side of the body and a loss of sensation (hemianesthesia [ninds.nih.gov]
This condition results in weakness or paralysis on one side of the body (hemiparaplegia) and a loss of sensation on the opposite side (hemianesthesia). Brown-Sequard syndrome most commonly occurs in the the thoracic spine (upper and middle back). [ebi.ac.uk]
The spinal cord is damaged but is not severed.Symptoms include weakness/paralysis (hemiparaplegia) to one side of the body and a loss of sensation (hemianesthesia) to the opposite side of the body. [patientslikeme.com]
Brown-Sequard syndrome (BSS) is a rare neurological disorder characterized by a lesion on the spinal cord which results in weakness or paralysis (hemiparaplegia) on one side of the body and a loss of sensation (hemianesthesia) on the opposite side. [web.archive.org]
- Neglect
Imaging studies might also be important, although not necessary, especially when additional injuries are suspected, like for example intraabdominal injuries or those resulting from bone fractures which are frequently neglected during physical examination [symptoma.com]
He neglected to see a doctor until the paresis aggravated and extended to ipsilateral lower limb. Acute presentation of cervical discogenic BSS after a pure trauma was even rarer. [journals.lww.com]
Workup
Physical examination is the key procedure to diagnose BSS, although laboratory studies might be useful to confirm the results, especially in the cases of nontraumatic epidoses like infections and tumors. Imaging studies might also be important, although not necessary, especially when additional injuries are suspected, like for example intraabdominal injuries or those resulting from bone fractures which are frequently neglected during physical examination. In particular, MRI might help understand the structure of the point damaged or identify nontraumatic transformations like tumors. CT scanning, instead, might be very useful to identify the injury on nerve tissue around the damaged point of the spinal cord [11] [12]. Other imaging studies include angiography, to identify vascular malformations, and nuclear medicine scans, to identify infections or inflamed states responsible for the episode of BSS. Lumbar puncture is used only for specific etiologies, like multiple sclerosis, tumor, or tuberculosis, where it is usually coupled with the laboratory analysis of cerebral spinal fluid.
X-Ray
- Cervical Spine X-Ray
Routine screening x-ray showed a unilateral facet dislocation at C5-6. A drawing of the lateral cervical spine x-ray is shown at left. There is an interruption of the normal smooth anterior and posterior curve of the cervical vertebra, shown in red. [en.wikibooks.org]
Pleura
- Pleural Effusion
Additional pulmonary complications include pneumonia, pulmonary embolism, pleural effusion, pneumothorax, and mucus plug. Gastrointestinal Complications include ileus, constipation, pancreatitis, abdominal rigidity, and stress ulceration. [neuropedia.net]
Treatment
Emergency care of BSS is performed through a thorough physical examination of the injury, which should be followed by a cervical spine/dorsal spine immobilization, with the exception of neck, before the beginning of physical therapy. It is essential to perform a complete and detailed physical examination, with the aid of imaging studies, because the sensory loss might make the identifications of the injury(ies) much more difficult.
The goals of physical therapy is to maintain strength in the intact muscles and assure proper movement in the joints. Furthermore, patients should be positioned in an appropriate way in order to avoid subsequent skin breakdown, improve respiratory function and achieve early mobilization to increase upright position tolerance. Recovery of functional movement has to be performed with a progressive and gradual manner, starting from bed mobility and going on with wheelchair mobility and prostheses-aided ambulation. As neurologic recovery continues even after discharge, therapy should be performed in an outpatient setting as well, coupled with home exercise programs to maintain patient’s strength, flexibility, and balance.
Many studies have demonstrated better clinical outcomes with patients undergoing corticosteroid administration, drugs which are well known for their anti-inflammatory properties based on their capability of modifying the immune response to external stimuli. Other drugs used to manage the signs and symptoms of BSS include antibiotics, antispasmodics and pain medications.
Prognosis
The prognosis is relatively good, with many severe cases showing significant motor recovery. This is very fast in the first two months, but slows down and continues for at least one year, even though there are cases in which it might even take up to two years. The classical healing pattern shows first of all the recovery of the ipsilateral proximal extensor muscles and distal flexor muscles, then the recovery from the weakness in the extremity affected by sensory loss, and recovery of voluntary motor strength within 1 to 6 months.
The potential long-term complications of BSS are very similar to those seen in other types of spinal cord injuries, especially problems related to the lower extremities causing difficult ambulation, although the final clinical outcome appears to be much better [10].
Etiology
The most common cause of BSS is a violent trauma [2] with a penetrating mechanism, which results in the injury of just one side of the spinal cord. Classical example of events causing a damage like this include stab and gunshot wounds, or fractures resulted from a violent accident [3] [4]. Very frequent are also the assaults with a pen and injuries from blowgun darts [5].
The nontraumatic causes include frequent events and conditions such as tumor, disk herniation [6], multiple sclerosis, ligamentum flavum ossification [7], meningitis, and infection by some viruses like herpes zoster and herpes simplex. Rarer, but not less effective as etiological factor, is also chiropractic manipulation [8] [9].
Epidemiology
In the US there is no database which records cases of Brown-Séquard syndrome due to traumatic and nontraumatic events and its incidence is still unknown. To have a raw approximation, it might be useful to know that the incidence of spinal cord trauma in USA is around 12,000 cases per year, 2-4% of which are supposed to result in BSS. No data about the international incidence of spinal cord trauma are available.
Spinal cord trauma events appear to be more frequent in the white population than in the other ethnic groups. The same tendency can be seen in males, where the frequency of spinal cord events is much higher than in females. In any case, these data represent the traumatic injuries and might have no relation with nontraumatic ones. The average age of individuals affected by BSS is around 40 years [10].
Pathophysiology
The lesion created after an accidental event might affect the upper motor neuron pathway of the corticolspinal tract, the dorsal columns, and the spinothalamic tract. The clinical consequences depend on the neuronal system that has been damaged.
The main clinical sign resulting from a damage of the upper motor neuron pathway is spastic paralysis on the ipsilateral side, which is coupled with a flaccid paralysis of the muscles at the level of the lesion itself. Ipsilateral loss of vibration and proprioception are the main clinical signs of the damage of the two dorsal columns, fasciculus gracilis or fasciculus cuneatus. Loss of pain and temperature sensation to the other side of the lesion, instead, are the classical clinical signs of the damage to the spinothalamic tracts. These usually occur a few segments below the lesion, because spinothalamic tracts enter the cord and travel ipsilaterally a few segments before passing to the other side of the cord itself.
Prevention
Since the etiology is mainly made of accidental events, no prevention plan can be provided.
Summary
Brown-Séquard syndrome (BSS) is also known as Brown-Séquard's hemiplegia, Brown-Séquard's paralysis, hemiparaplegic syndrome, hemiplegia et hemiparaplegia spinalis, and spinal hemiparaplegia. BSS was first described during the 1840s by Dr Charles-Édouard Brown-Séquard, whose name then became the name of the condition itself. The ipsilateral hemiplegia with controlateral pain and temperature sensation is the result of the criss-cross pattern of the nerve fibers in the spinal cord.
Being an incomplete lesion, the clinical presentation shows a variety of neurological signs whose severity ranges from mild to severe. The appearance of a pure form of BSS, characterized by a perfect hemisection of the cord, is rarely seen. More common, instead, are the less pure forms with a clinical picture characterized by some signs and symptoms of BSS, plus additional ones which are not specific of this injury. This form of BSS is called Brown-Séquard–plus syndrome [1].
Patient Information
Brown-Séquard syndrome (BSS) is a type of incomplete spinal cord lesion, which is characterized by the paralysis and loss of proprioception on the body side of the injury and loss of pain and temperature sensation on the opposite one. Being an incomplete lesion, BSS shows a variety of neurological signs whose severity ranges from mild to severe.
The most common cause is a violent trauma with a penetrating mechanism which results in the injury of just one side of the spinal cord. Classical example of events causing a damage like this include stab and gunshot wounds, or fractures resulted from a violent accident. Very frequent are also the assaults with a pen and injury from a blowgun dart. The nontraumatic causes include conditions such as tumor, disk herniation, multiple sclerosis, ligamentum flavum ossification, meningitis, and infection by some viruses like herpes zoster and herpes simplex.
The prognosis of BSS is relatively good, with many severe cases showing significant recovery. The potential long-term complications are very similar to those seen in other types of spinal cord injuries, especially problems related to the lower extremities causing difficult ambulation, although the final clinical outcome appears to be much better.
Treatment is performed through a thorough physical examination of the injury, which should be followed by a cervical spine/dorsal spine immobilization, with the exception of neck, before the beginning of the physical therapy. Many studies have demonstrated better outcomes with patients undergoing corticosteroids administration, a group of drugs which are well known for their anti-inflammatory properties based on their capability of modifying the immune response to external stimuli. Other drugs used to manage the signs and symptoms of BCC include antibiotics, antispasmodics and pain medications.
References
- McCarron MO, Flynn PA, Pang KA, et al. Traumatic Brown-Séquard-plus syndrome. Arch Neurol. Sep 2001;58(9):1470-2.
- Ceruti S, Previsdomini M; Traumatic Brown-Sequard syndrome. J Emerg Trauma Shock. 2012 Oct;5(4):371-2. doi: 10.4103/0974-2700.102421.
- Musker P, Musker G. Pneumocephalus and Brown-Sequard syndrome caused by a stab wound to the back. Emerg Med Australas. Apr 2011;23(2):217-9.
- Mac-Thiong JM, Parent S, Poitras B, Joncas J, Labelle H. Neurological Outcome and Management of Pedicle Screws Misplaced Totally Within the Spinal Canal. Spine (Phila Pa 1976). Jul 18 2012.
- Moin H, Khalili HA. Brown Séquard syndrome due to cervical pen assault. J Clin Forensic Med. Apr 2006;13(3):144-5.
- Urrutia J, Fadic R. Cervical disc herniation producing acute Brown-Sequard syndrome: dynamic changes documented by intraoperative neuromonitoring. Eur Spine J. Jun 2012;21 Suppl 4:S418-21.
- Chen PY, Lin CY, Tzaan WC, et al. Brown-Sequard syndrome caused by ossification of the ligamentum flavum. J Clin Neurosci. Sep 2007;14(9):887-90.
- Lipper MH, Goldstein JH, Do HM. Brown-Séquard syndrome of the cervical spinal cord after chiropractic manipulation. AJNR Am J Neuroradiol. Aug 1998;19(7):1349-52.
- Domenicucci M, Ramieri A, Salvati M, Brogna C, Raco A. Cervicothoracic epidural hematoma after chiropractic spinal manipulation therapy. Case report and review of the literature. J Neurosurg Spine. Nov 2007;7(5):571-4.
- McKinley W, Santos K, Meade M, et al. Incidence and outcomes of spinal cord injury clinical syndromes. J Spinal Cord Med. 2007;30(3):215-24.
- Parmar H, Park P, Brahma B, et al. Imaging of idiopathic spinal cord herniation. Radiographics. Mar-Apr 2008;28(2):511-8.
- Miranda P, Gomez P, Alday R, et al. Brown-Sequard syndrome after blunt cervical spine trauma: clinical and radiological correlations. Eur Spine J. Aug 2007;16(8):1165-70.