Neurology and Neurosurgery/Incomplete Spinal Cord Injuries/Brown-Séquard syndrome

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Brown-Sequard syndrome is a rare yet classic type of spinal cord injury. Due to its rare occurrence most of the discriptions from the literature are in the form case studies.

Representative cases[edit | edit source]

Case 1: Knife wound to the spinal cord[edit | edit source]

MRI of the chest showing a collapsed left lung on the right side of image (w:pneumothorax) typically seen in a knife wound to the chest.

A 25-year-old male presented to the emergency room, transported by ambulance. He was stabbed in the posterior mid-thoracic region. Admission blood pressure was 90/60. Pulse was 120 per minute. He was ashen and appeared to be in shock. Two peripheral IVs were started, and he was typed and crossed for 6 units of blood. Chest x-ray showed a left sided pneumothorax (collapsed lung) and associated left sided hemothorax (bleeding in the chest). Examination revealed a 2 inch stab wound to the back at about the level of the T6. Neurologic exam revealed complete loss of left sided motor function below T6 and loss of sensation on the right below T6. Admission hemoglobin was 9.6 gms% and hematocrit is 37. He began to show signs of respiratory distress. The patient was intubated (breathing tube) and placed on an assisted ventilation respirator. The surgery resident placed a chest tube on the left side, and bright red blood immediately flowed from the chest tube. The chest was opened on an emergency basis. Lacerations to the left lung and aorta were encountered and repaired. Neurosurgery explored the spinal cord at the level of T6 and found a laceration of the cord on the left side at T6. The patient’s life was saved, but his neurologic deficit is permanent, due to the laceration of the spinal cord at the T6 level. This is a typical case presentation of Brown Sequard syndrome. [1] [2] [3]

Similar cases have been reported except without a demonstrable laceration of the spinal cord. Sometimes, a hematoma without laceration to the spinal cord can cause a Brown-Sequard syndrome. After the patient is stable, a neurosurgical procedure consisting of a laminectomy and evacuation of the hematoma at the effected level can be carried out. Many times, this can lead to a complete neurologic recovery. [4] [5] [6] This case represents the rare situation whereby a full recovery can be accomplished when the spinal cord is not actually lacerated, and the Brown Sequard syndrome is caused by bleeding and pressure from a hematoma. Quick evacuation of the hematoma is necessary to achieve a good result. [7] [8]

In a related case, the authors described the history of a patient who was stabbed in the back of his neck with a knife and who later presented with a Brown-Séquard syndrome attributable to cervical spinal cord damage. Myelography and CT revealed a compressive extradural lesion shown at exploratory operation to be a loculus of cerebrospinal fluid (CSF). The loculus had formed as a consequence of leakage of CSF through a dural tear caused by the knife. Evacuation of this loculus resulted in some neurological improvement. The necessity of obtaining a clear history and of performing a thorough clinical examination is explained. The need to admit patients in whom stab wounds of the neck have transgressed subcutaneous fat is reiterated. Early referral to a neurosurgeon is advised for those patients with neurological deficits. [9] [10]

Case 2: Diving accident[edit | edit source]

Drawing of lateral cervical spine fracture showing a wedge fracture of C6 with posterior dislocation of C5 on C6.

An 18-year-old male was brought to the emergency room by his friends with a history of diving into a local pond. He came to the surface and was noted to be unable to move his arms or legs. His friends reported that he had a head injury to the admitting clerk at the hospital. They said the water was shallow and he hit his head on the bottom. Because of what they perceived to be a head injury, they determined he was paralyzed. Careful examination revealed a motor loss below C6 on the right and a loss of sensation on the left. He had preservation of motor function on the left, but is was noted to be weak. Examination of the head revealed no focal neurologic signs. He was alert and awake, although anxious and sweating. His blood pressure was 80/40 and pulse was 160 and regular. Two peripheral IVs were started and rehydration was begun. He had no memory of the event, other than hitting his head and experiencing immediate pain in the neck. A drawing of his lateral cervical (neck) x-ray is shown at right. He was diagnosed with a wedge compression fracture of C6 with disruption of the posterior stabilizing elements of C5-6 and C6-7. There was a backwards (retrolisthesis) dislocation of C5 on C6. The normal anterior and posterior curves of the vertebra (shown in red) have been disrupted, indicating a fracture dislocation. He was diagnosed with Brown-Sequard syndrome. The fracture and dislocation of the cervical spine was determined to be unstable, and manipulation risked further loss of function by damaging the spinal cord to a greater extent. Traction was placed, and later and extensive anterior-posterior fusion was carried out between C4 and T1. After about 6 months, the patient had regained about 40% of his motor function and about 60% of sensation. His deficits remained permanent, although he could eventually walk with braces and hand-arm crutches. The hypotension (low blood pressure) observed in the emergency room was caused by blood pooling in the legs secondary to spinal shock. [11] [12] [13] [14] [15]

Case 3: Automobile accident[edit | edit source]

MRI images showing C6 fracture dislocation with cord compression.

A 17-year-old girl was riding with her friends in a convertible car. Several of them were sitting on the top of the back seat without seatbelt restraint. The car was traveling in excess of 70 miles an hour, when the driver made a sudden swerving in turn. Several of the passengers were thrown from the car. One was noted to be unconscious at the scene. An ambulance was called. Upon admission to the hospital she was noted to have a laceration over the top of the head. She had lost a large amount of blood. She was wavering in and out of consciousness. Blood pressure was 90/70 with a pulse of 120. Peripheral IVs were started with rehydration carried out. Examination revealed an epidural bleed in the right side of the brain. Screening x-rays showed a C6 fracture dislocation. She was placed in traction. No other injuries were noted. Abdomen and chest were within normal limits. Since the epidural bleed was significant, it was surgically evacuated the night of admission. On the third day, she regained consciousness and was noted to have lost motor function on the left side of her body and loss of sensation on the right. A diagnosis of Brown-Sequard syndrome was made. Surgical stabilization of the cervical fracture was planned. This was carried out on the sixth hospital day, with an anterior-posterior fusion from C5 to T1 and stabilization of the C6 fracture. There was no improvement in her neurologic status after the surgery. At two weeks, she was noted to have progressed to a complete tetraplegia (paralysis of both arms and legs). She developed respiratory distress. A tracheotomy was performed and she was placed on a ventilator. On the 26th hospital day, she was noted to be in respiratory distress, blue and struggling. A diagnosis of pulmonary embolism was made. Her course was downward. On the 40th hospital day, she died from sepsis secondary to pneumonia. This case represents an unfortunate outcome of a severely injured patient. It illustrates that a Brown-Sequard syndrome can progress to a complete paraplegia, despite adequate treatment. [16] [17] [18] [19] [20] [21] [22]


Case 4: Degenerative arthritis[edit | edit source]

X-ray showing advanced degenerative arthritis of the cervical spine with osteophyte formation, which can bruise the spinal cord in a fall.

A 72-year-old woman experienced dizziness while taking a bath in her home. She fell in the tub, experiencing a contusion to the back of the head and neck. Her daughter heard the fall and called an ambulance. At the hospital, she was found to have partially lost motor function on the right below C6 with almost total paralysis of the right arm and partial paralysis of the right leg. She had lost sensation in the left arm, and had patchy loss of sensation in the left leg. X-ray revealed advanced degenerative arthritis of the cervical spine. MRI demonstrated a contusion of the spinal cord on the right at the C6 level. The cervical ridges secondary to the degenerative arthritis are called osteophytes, and in this case, caused a contusion (bruise) to the spinal cord when she fell in the bath. The diagnosis of Brown-Sequard Syndrome was made. She reported that she had pain in the neck for several years, although during the last few years, her pain had been less. She had seen a neurosurgeon ten years earlier, who had recommended a discectomy and fusion of C4-5 and C5-6. She had declined his recommendation. After a 6 week stay in the hospital, she was able to be discharged to a nursing facility With expectant therapy and physical therapy, she slowly gained back function over the next six months. Recovery was incomplete. After six months, she was able to ambulate with assistance and a walker. Originally, bowel and bladder function had been interrupted. These returned at six weeks after the injury.

In a Japanese study of the postoperative prognosis of the hemihypalgesia (decreased sensation) in patients with Brown-Séquard-type myelopathy (BSM) caused by cervical lesions, it was discovered that all continued to have partial or complete loss, even if surgery was performed. They evaluated the prognosis of BSM in 16 (seven ossification of the posterior longitudinal ligament [OPLL], 5 cervical spondylosis [CS], and 4 disc herniation patients) out of 233 surgically treated patients with cervical diseases. The mean follow-up duration was 2 years and 11 months. After surgery, none of these patients showed complete resolution of hemihypalgesia, although the most had an improvement in symptoms. In the Brown Sequard Myelopathy series, OPLL occurred most frequently and the anterior element compressing the spinal cord existed most frequently in the central area of the vertebra. Postoperative improvement in the motor function of the legs in the disc herniation group was significantly better than in the OPLL and CS groups There were no significant differences in the functional prognosis between the BSM and non-BSM patient groups. It was concluded Brown Sequard Myelopathy patients can expect almost the same functional outcome as non-Brown Sequard Myelopathy patients, with the exception of the disappearance of hemihypalgesia. [23]

Case 5: Unilateral facet dislocation[edit | edit source]

Drawing of a lateral cervical spine x-ray showing a unilateral facet dislocation, with a <25% forward slipping of C5 on C6.

A 25 year old rode his motorcycle into the side of a truck at an intersection. He lost consciousness and was noticed to have an open fracture of the left distal tibia and fibula above the level of the ankle. The neck was immobilized in the usual fashion. He was transported to the hospital. Exam showed motor paralysis of the left arm and leg with loss of sensation on the right side of his body. 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. The forward slipping of C5 on C6 of less than 25% the width of the vertebra suggests the diagnosis of unilateral facet dislocation. It was confirmed with an MRI. Neurosurgery was consulted, and traction applied to the head and neck. Over a period of five days, the unilateral facet dislocation was reduced. It was thought to be unstable, so a posterior fusion was carried out. The patient gained about 80% of his motor function and all of his sensation over the next six months. The open tibial-fibula (leg) fracture was treated with an external fixator afer irrigation. There was some skin loss over the fracture with an associated osteomyelitis (bone infection). This required several more surgeries, including debridement of the infected bone and skin grafting. Healing of the tibial and fibular fractures eventually occurred after 18 months. After healing, he still had some difficulty with ambulation and found it necessary to walk with arm crutches. He was markedly limited in his ability to walk or stand for any length of time. He had trouble finding employment and sought permanent disability because of his significant impairments. [24] [25] [26]

Case 6: Gunshot wound[edit | edit source]

A case of stray bullet injury in a child was reported who presented with Brown-Sequard syndrome and CSF leak from the wound at the nape of neck. Patient was assessed by plain radiography and CT scans showing bullet lying in the cervical spinal canal under the C1 and C2 laminae. Laminectomy at C1/C2 level was done and bullet was carefully removed. Patient improved neurologically and CSF discharge stopped. The case report indicated the atypical neurological presentation and possibility of survival in high cervical spinal firearm injury. [27]

A patient with a gunshot wound to the spinal cord with an incomplete neurologic deficit presented to the emergency room where the neurologic examination revealed a combination of a central cord syndrome type injury and the Brown-Séquard Syndrome. It was speculated by the examining doctors that the Brown-Séquard portion of the syndrome was caused by compression of tracts within the spinal cord caused by the mass of the bullet and the central cord injury was produced by the kinetic energy of the bullet during penetration into the spinal canal. They conclude that with incomplete neurologic lesions following gunshot wounds the bullet be removed. [28]

Case 7: Cavernous malformation[edit | edit source]

MRI of bleeding into a cavernous sinus within the spinal cord

A 17-year-old boy presented with diabetes insipidus and delayed puberty. The authors reported the first case of demonstrated de novo formation of an intramedullary cavernous malformation (CM) following spinal radiation therapy. Analysis of the literature has suggested that cavernous malformations (CMs) of the brain may be an acquired pathogenesis and a dynamic pathophysiological composition, with documented appearance of new lesions in familial cases and following radiotherapy Evaluation of endocrine levels revealed hypopituitarism, and magnetic resonance (MR) imaging demonstrated an infundibular mass. The patient underwent a pterional craniotomy and removal of an infundibular germinoma. The MR image of the spine demonstrated normal results. The patient received craniospinal radiation therapy and did well. He presented 5 years later with acute onset of back pain, lower-extremity weakness and numbness, and difficulty with urination. An MR image obtained of the spine revealed an intramedullary T-7 lesion; its signal characteristics were consistent with a cavernous malformation. The patient was initially managed conservatively but developed progressive myelopathy and partial Brown-Séquard syndrome. Although he received high-dose steroids and bed rest, his symptoms worsened. He underwent a costotransversectomy and excision of a hemorrhagic vascular lesion via an anterolateral myelotomy. Pathological examination confirmed features of a CM. The patient has done well and was walking without assistance within 4 weeks of surgery. De novo genesis of CMs may be associated with prior radiation therapy to the spinal cord. [29] Radiation therapy can cause cavernous malformations by destroying the malignant tissue, leaving an empty space which fills with blood or serous fluid. If these enlarge progressively, increasing pressure on the surrounding neurologic tissue causing various deficits in nerve patterns. In this case, the deficit fit the pattern of a partial Brown-Sequard syndrome. See infra for another report of cerebral-spinal germinoma causing a Brown Sequard syndrome.

Case 8: Germinoma[edit | edit source]

Spinal cord germinoma causing Brown Sequard syndrome has been reported in the Japanese literature. [30]

Testicular seminoma showing pathology similar to a germinoma.


A 29-year-old man presented with diabetes insipidus and cerebellar ataxia who developed spinal cord swelling 15 years after the onset. He was well until 14 years of the age when he noted dizziness. Two years after there was an onset of gait disturbance and slurred speech. He also noted polydipsia and polyuria. He was evaluated at the neurosurgery service of our hospital when he was 17 years of the age. Neurologic examination at that time revealed memory loss, horizontal nystagmus, cerebellar ataxic gait, dysmetria and decomposition more on the left. Cranial CT scan revealed a mass lesion involving the left subthalamic region and the head of the caudate area. Spinal fluid was unremarkable, however, human chorionic gonadotropin was increased to 27 mIU/ml. He was treated by radiation therapy (3,000 rads for total brain area and 5,460 rads for focal region). His CT scan and memory loss improved, however, cerebellar ataxia was unchanged. Three years after the radiation, he started to show choreic movement in his neck and left upper extremity.

Most seminomas originate in the testicle, however some can arise de novo in the brain as in this case.

He was admitted to our service on August 14, 1995 when he was 29 years of the age. On admission, he was alert but disoriented to time; calculation was also poor. Higher cerebral functions were intact. The optic fundi were normal without papilledema. Visual field appeared intact. Gaze nystagmus was observed in all the directions, but more prominent in the horizontal direction. Speech was slurred. Otherwise, cranial nerves were unremarkable. Motor wise, he showed marked truncal and gait ataxia; he was unable to walk because of ataxia. Muscle atrophy and marked weakness was noted in both upper extremities more on the left side. Deep tendon reflexes were diminished in the upper extremities but active in the lower extremities. He was polyuric; urinary specific gravity was low. Spinal fluid contained 6 cells/cmm and 113 mg/ dl of protein; Queckenstedt was positive. MRI revealed swelling of the cervical cord; in addition, the entire cervical region and the medullar oblongata appeared as high signal intensity areas. No mass lesion was noted in the supratentorial structures but the third ventricle was markedly enlarged. Surgical biopsy was performed on the cervical lesion. The patient was discussed in neurologic CPC, and the chief discussant arrived at the conclusion that the patient had germinoma with syncytiotrophoblastic giant cells in the diencephalic region which appeared to have been cured by radiation therapy; he thought that the cervical lesion was the seeding of germinoma. Cerebellar ataxia was ascribed to the remote effect of germinoma. Most of the participants thought that the original tumor was germinoma and the cervical lesion was its spread. Some participants thought that his ataxia was caused by germinoma cells involving the medulla and the inferior cerebellar peduncles. Histologic observation of the biopsied tissue from the spinal cord revealed the typical two cell patterned germinoma. Most of the tumor cells were not stained for an antibody against HCG, but some tumor cells were positively stained. Germinoma is very radio-sensitive; this patient showed T2 high signal lesion involving the medulla oblongata and cervical cord continuously. Probably, tumor cells in the lower brain stem escaped radiation, and gradually spread to the spinal cord over many years. At the time of operation, the surface of the spinal cord was free from tumor cells. Therefore, tumor cells invaded the spinal cord continuously from the medulla oblongata. He was treated with cervical radiation, and his neurologic as well as radiologic findings showed marked improvement.

Multiple Sclerosis[edit | edit source]

Magnetic Resonance Image with enhancement showing multiple sclerosis of the cervical spine.

Multiple Sclerosis presenting as Brown Séquard syndrome. A case of probable multiple sclerosis (MS) in the cervical cord without brain involvement, mimicking a tumor has been reported. The patient had a progressive left Brown-Séquard syndrome. The diagnosis was suggested by MRI. In spite of intensive corticotherapy, the neurological status worsened, in parallel with the radiological findings. Biopsy excluded an intramedullary tumour and supported the diagnosis of MS. [31] The Brown-Séquard syndrome as spinal hemiplegia with contralateral sensory deficits has been related to a variety of underlying diseases. We describe the case of a 35-year-old right-handed white female presenting with a Brown-Séquard syndrome as the first and sole symptom of multiple sclerosis, underscoring the importance of multiple sclerosis as a rare condition for the Brown-Séquard syndrome. (MS) is an idiopathic inflammatory demyelinating disease of the CNS. Patients with MS commonly present with an individual mix of neuropsychological dysfunction, which tends to progress over years to decades. The diagnosis of MS is based on a classic presentation (ie, optic neuritis, transverse myelitis, internuclear ophthalmoplegia, paresthesias) and on the identification of other neurologic abnormalities, which may be indicated by the patient history and exam. Typical findings on an MRI also help establish a diagnosis of MS. By convention, the confidence in the diagnosis of MS is described as definite, probable, or possible MS. It includes a classification with respect to clinical presentation, which correlates somewhat with the prognosis and is useful in clinical trials. About 70% of patients present with the more favorable relapsing-remitting (RR) type, which is characterized by acute exacerbations with full or partial remissions. Therapy for those with chronic progressive MS is less satisfying than for those with RR. Patients with an inflammatory component may respond to corticosteroids, beta-interferons, or intravenous cyclophosphamide (Cytoxan), and one study found modest impact on disease progression when the patients were given a low dose of methotrexate. Mitoxantrone is sometimes recommended for patients with rapidly progressive symptoms.

MS is regarded as an autoimmune disease. Most of what is known about MS is derived from its model in animal research, which is experimental allergic encephalomyelitis. The pathologic hallmark of MS is multicentric, multiphasic CNS inflammation and demyelination. Originally, each MS lesion was thought to evolve through episodes of demyelination and remyelination into a chronic burned-out plaque with relative preservation of axons and gliosis. Thus, the neuropsychological dysfunction occurred, despite an essentially intact neural network, until late in the disease course. However, recent studies have demonstrated that axonal transections do occur during acute exacerbations; furthermore, axonal damage, as measured by magnetic resonance spectroscopy, was found to correlate with clinical disability. For unclear reasons, lesions characteristically involve the optic nerve and periventricular white matter of the cerebellum, brain stem, basal ganglia, and spinal cord. Identifying MS lesions in gross specimens is difficult, as is identifying MS lesions in gray matter on radiographic images; hence, the predilection for white matter may not be disease related. The peripheral nervous system rarely is involved. [32] This case represents an unusual presentation of Multiple Sclerosis because it occurred in the spinal cord without evidence of pathology in the central nervous system. [33] [34]

Hematoma secondary to blood thinner[edit | edit source]

52-year-old man on blood thinner developed motor paralysis below C7 on the left and loss of sensation and proprioception below C7 on the contralateral side was reported. MRI showed an epidural hematoma at C7-T1. A cervical laminectomy with accomplished on an emergency basis with evacuation of the hematoma, which was outside the spinal cord, but pressing on it. Post-operatively, there was a complete resolution of neurologic function. [35] [36] [37] [38] [39][40] [41] [42] [43]

Spinal cord infarction[edit | edit source]

Posterior spinal cord infarction presenting Brown-Séquard syndrome has been reported in Japan. A 56 year-old-woman with spinal cord infarction (loss of blood supply) presented to the hospital. She experienced left-sided girdle pain without precipitating symptoms and she developed monoparesis (paralysis of one leg) of her left leg and urinary retention. She also presented the segmental loss of total sensations in the Thoracic 10-11 area of the left trunk, the disturbance of position and vibration senses in the left leg and the disturbance of pain and temperature senses in the right leg. T2-weighted MRI imagings showed high signal intensity lesion in the left half of the spinal posterior column at Th9-10 vertebral levels. Somatosensory evoked potentials confirmed that the loss of position and vibration senses was unilateral. Though she became able to walk with canes two months later, her sensory disturbance showed no improvement. This is a rare case of unilateral posterior spinal cord infarction presenting Brown-Séquard syndrome. [44]

Syringomyelia[edit | edit source]

MRI demonstrating syringomyelia of the cervical spine, shown in the gray linear mass within the spinal cord.

Syringomyelia is a pathologic condition which is caused by a fluid filled cavity within the center of the spinal cord. The sac is called a syrinx. These can enlarge, causing a myriad of neurologic presentations, including Brown Sequard syndrome and its variants. Two cases have been reported of syrinx associated with an intramedullary metastasis. The first presented with hyperaesthesia in the right arm and bilateral pyramidal leg weakness, the second with a partial right sided Brown Sequard syndrome with a sensory level at D6 (T6) . Possible mechanism are discussed. . [45] A third patient having intramedullary spinal cord metastasis associated with syringomyelia has been reported. The diagnosis was confirmed by magnetic resonance imaging, in a patient who had poorly differentiated carcinoma of the lung. The patient responded to treatment with steroids and radiotherapy, with complete resolution of neurologic symptoms and syringomyelia. Intramedullary spinal cord metastasis is relatively rare. [46]

Based on examination of 19 patients with syringomyelia and on the related literature, including the differential diagnosis, postoperative evaluation and the dynamics of CSF and cyst fluids, using magnetic resonance imaging (MRI), the following conclusions can be drawn: 1. MRI is the preferred method of investigation for diagnosis and differential diagnosis of syringomyelia. 2. Using MRI, it is possible to study fluid flow in the vertebral canal and the movements of the syrinx fluid. The data are as yet limited, but in the future will form an important contribution to our understanding of the pathogenesis of syringomyelia and to the choice of a treatment method. 3. MRI is important for the postoperative follow-up of patients with syringomyelia, including tracking the cyst and detecting complications.[47] Syringomyelia causes progressive myelopathy. Most patients with syringomyelia have a Type I Arnold-Chiari malformation of the cerebellar tonsils. Pathophysiological mechanisms underlying the progression of syringomyelia are associated with the Chiari Type I malformation which are frequently seen with the condition. In a study of 20 adult patients with both Arnold-Chiari Type I malformation and symptomatic syringomyelia, testing before surgery included the following: clinical examination; evaluation of anatomy by using T1-weighted magnetic resonance (MR) imaging and evaluation of the syrinx. Compared with healthy volunteers, before surgery, the patients had decreased anteroposterior diameters of the ventral and dorsal CSF spaces at the foramen magnum. The maximum syrinx diameter decreased on MR imaging in all patients. Cine MR imaging documented reduced velocity and flow of the syrinx fluid. Clinical symptoms and signs improved or remained stable in all patients, and the tonsils resumed a normal shape. The progression of syringomyelia associated with Arnold-Chiari Type I malformation is produced by the action of the cerebellar tonsils, which partially occlude the subarachnoid space at the foramen magnum and act as a piston on the partially enclosed spinal subarachnoid space. This creates enlarged cervical subarachnoid pressure waves that compress the spinal cord from without, not from within, and propagate syrinx fluid caudally with each heartbeat, which leads to syrinx progression. The disappearance of the abnormal shape and position of the tonsils after simple decompressive extraarachnoidal surgery suggests that the Arnold-Chiari Type I malformation of the cerebellar tonsils is acquired, not congenital. Surgery limited to suboccipital craniectomy, C-I laminectomy, and duraplasty eliminates this mechanism and eliminates syringomyelia and its progression without the risk of more invasive procedures. [48]

Renal Cell Carcinoma[edit | edit source]

The diagnosis of a metastatic lesion to the spinal cord can be made by biopsy, such as this clear cell carcinoma of the kidney.

Any metastatic cancer to the spinal cord can cause a Brown-Sequard syndrome or its variants. In clinical practice, these are rare. A patient presented with a solitary neoplasm of the intramedullary spinal cord. The tumor caused findings of Brown-Séquard syndrome and was the initial presentation of widely metastatic renal cell carcinoma (RCC). Metastasis of the intramedullary spinal cord has been uncommonly reported and is extremely rare as the initial sign of a disseminated carcinoma. The tumor was resected, and the patient's neurologic deficits slowly improved. The histopathology suggested an epithelioid neoplasm. However, despite expert review, the diagnosis was nonspecific, and the tumor appeared benign. It soon recurred in the spinal cord, and repeat imaging studies showed numerous new metastases and a primary renal neoplasm. Biopsy and a repeat histopathologic analysis confirmed RCC, and the patient died of disseminated disease within another 2 months. Despite being uncommon, spinal cord metastases should be considered in some patients before surgery because it may expedite diagnosis, mitigate the need for surgery, and improve the quality of life for these patients. Clinical factors suggesting metastasis include a personal or family history of malignancy or conditions predisposing to it, the presence of multiple tumors in the spinal cord or elsewhere, nonspecific constitutional symptoms, such as weight loss or decreased appetite, and, specifically for RCC, an abnormally increased hematocrit. [49] In another case, the authors reported of a 70-year-old woman who developed a Brown-Sequard-syndrome within 6 weeks caused by an intramedullary spinal cord metastasis of an occult renal cell carcinoma. Intramedullary metastases are rare and represent only 4-8.5% of central nervous system metastases. An important feature of intramedullary metastases is the rapid progression of neurological deficits which necessitates rapid treatment. There are only eight earlier reports of intramedullary metastasis due to renal cell carcinoma [50] [51] [52] [53] [54] [55] [56] The authors reported that to the best of their knowledge this was the first report on a patient in whom symptoms from the metastasis of a renal cell carcinoma preceded the detection of the primary tumor. This report presents the clinical, neuroradiological and histopathological findings of an intramedullary metastasis of a renal cell carcinoma and provides an overview of the literature on intramedullary spinal cord metastases. [57] However, there have been other cases where the presence of a metastasis causing a Brown Sequard syndrome, or a variation of it was the first sign of a renal cell carcinoma. In a Spanish case, a 69 year-old male without known antecedent presented with a clinical loss of distal force in his lower limbs during the last two months. Magnetic resonance imaging (MRI) showed an intramedullary spinal cord mass at the level of the medullaris cone. The patient was operated on; the histological diagnosis was clear cells carcinoma suggestive of metastasis from an unsuspected renal tumor that was later confirmed with an abdominal CT. When the patient's state is good, surgery can correct the neurological deficit produced by an intramedullary spinal cord lesion. The neurological state of the patient improved after the intervention, and 14 months after surgery, he had no neurological deficit in the lower limbs. [58] [59]

Lung cancer[edit | edit source]

Biopsy demonstrating small cell carcinoma of lung (oat cell carcinoma) typically seen in metastatic lesions.

Four patients with intramedullary spinal cord metastases from small cell carcinoma of the lung (SCCL) were described, with emphasis on clinical presentation and treatment. All patients developed the Brown-Séquard syndrome due to intramedullary tumor in the cervical spinal cord, three within 2 mo after combined modality treatment using chemotherapy and radiotherapy. One patient presented with a Brown-Séquard syndrome and an extradural spinal cord compression from tumor. The radiological and cerebrospinal fluid findings are presented and discussed. Radiation treatment was administered to the involved segments of spinal cord in each patient. All patients responded; two for 3 and 7 mo, two for 3 and 4 wk, respectively. There was significant improvement in the quality of life for three of the four patients. [60] [61]

MRI of the thorax showing a lung cancer.

Intramedullary metastases are very rare. Four cases verified by autopsy were reported. The site of the primary tumor was the lung (three cases) and the breast (one case). One case was complicated by a Brown-Séquard syndrome. We found 96 cases of intramedullary metastases in the literature. The primary tumor sites were lung (49%), breast (14%), kidney (6%) and colo-rectal region (5%). Melanoma was noted in 6% and Hodgkin's disease in 4% of the cases. Other primary tumors comprised 9%. The intramedullary metastases were most commonly thoracic. [62] In another report, the authors reviewed records retrospectively from 1980 to 1993 to identify patients with histologically confirmed systemic cancer, clinical features of myelopathy, and either tissue-proven ISCM or abnormal neuroimaging findings consistent with ISCM. They identified 40 patients who fulfilled these criteria. In nine, ISCM was the initial presentation of cancer. Nineteen patients had lung primaries (small cell in 12). Twenty-one patients had pain, 35 had demonstrable sensory loss, 37 had weakness, and 25 had urinary incontinence at presentation. Nine patients had true Brown-Séquard syndrome and nine others had pseudo-Brown-Séquard syndrome. Median duration of symptoms at diagnosis was 28 days (range 3 days to 18 months). Thirteen patients had prior brain metastasis, nine had brain metastasis simultaneous with ISCM, and one had brain metastasis after ISCM; 11 had concomitant leptomeningeal metastases. Spinal magnetic resonance findings were abnormal in 30/30 patients, myelographic results were abnormal in 16/20, and eight had pathologic confirmation of ISCM. Thirty-five patients had radiotherapy and five had surgery; four were untreated or treated elsewhere. Median survival was 4 months for patients receiving radiotherapy and 2 months for patients not receiving radiotherapy. Eleven patients survived > 6 months. Twenty-three were ambulatory at ISCM diagnosis, and 21 were ambulatory at letest follow-up. We conclude that ISCM as the initial presentation of malignancy is not rare, and hemicord syndromes occur frequently. Although long-term survival is poor, treatment preserves ambulation in most patients still ambulatory at diagnosis. Focal radiotherapy is indicated in most patients. [63] [64] [65]

Tuberculosis[edit | edit source]

Typical chest x-ray in advanced tuberculosis.

A case from Korea reporting a Brown-Sequard syndrome secondary to tuberculosis of the spine in a systemic lupus erythematosis patient: A 25-year-old girl presented with progressive deterioration of right side weakness with decreased sensation on the left trunk. She had been treated with high dose steroid due to autoimmune thrombocytopenia for 2 months. Clinical, laboratory and immunologic studies revealed that she had systemic lupus erythematosus (SLE), MRI of spinal cord showed marginal contrast enhancing and fluid containing mass in the cord of the C5-6 level, suggesting intramedullary abscess. She underwent surgery of mass removal with biopsy. The pathologic findings from cord tissues revealed numerous acid fast bacilli (AFB) in necrotic tissues. After surgery and anti-tuberculous treatment, her neurologic symptoms were markedly improved with restoration of right side motor weakness. To our knowledge, this is the first case report of intramedullary tuberculosis in a patient with SLE. Since intramedullary tuberculosis may sometimes mimic neurologic complication of SLE itself, it may pose diagnostic and therapeutic confusion for clinicians. We report a case of spinal cord tuberculosis affecting C5, 6 level which was manifested as Brown-Séquard syndrome in a patient with SLE. [66]

A report from South Africa highlights reviewed cases of spinal tuberculosis with varying degrees of neurologic involvement. The incidence of tuberculosis (TB) is increasing in both developing and developed worlds, and children, in particular, represent a high-risk group for acquiring the disease. TB of the central nervous system is the most severe, life-threatening form of TB in infants and children. Approximately 10% of all patients with TB have central nervous system involvement. for this paper, the authoes selected four pediatric cases of unusual spinal TB that presented to their institution during the last 5 years. These include TB arachnoiditis, intramedullary spinal cord tuberculoma, TB spondylitis of the odontoid peg, and one child with tuberculous extradural abscess. TB may involve the intramedullary, extramedullary intrathecal compartment, or the extrathecal vertebral compartment in the form of an arachnoiditis, abscess, and spondylitis, respectively, often with unusual imaging presentations. [67]

Syphilis[edit | edit source]

A man aged 31 presented to hospital with acute onset of weakness in the legs, sensory loss, and disturbance of bladder and bowel function. Incomplete Brown-Séquard syndrome secondary to meningovascular syphilis of the spinal cord was diagnosed after serological tests for syphilis gave positive results. His condition was probably caused by endarteritis of the arteria radicularis magna of Adamkiewicz. He was treated with procaine penicillin 1.8 MIU intramuscularly once a day and probenecid 0.5 g by mouth three times a day for 21 days followed by physiotherapy and rehabilitation. Considerable neurological recovery was expected. To our knowledge this association has not been reported previously in detail in an English publication. [68]

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