NEUROLOGY AND NEUROSURGERY - Y. V. ALEKSEENKO - 2014

BRAIN AND SPINAL CORD TUMORS

Nervous system tumors represent one of the most pressing challenges in modern neurology and general medical practice. Over recent decades, we have witnessed significant advancements in Treatment options and improved outcomes for patients with nervous system neoplasms. This underscores Structure/19.html">The Importance of early recognition and The Need for physicians across various specialties to understand the algorithms for initial patient management. The primary objective at this stage is to promptly formulate a suspicion of a tumor-related CNS lesion and refer the patient to a neurologist or neurosurgeon for targeted diagnostic evaluation.

Among all neoplasms, Brain Tumors account for approximately 10%. In pediatric populations, they represent up to 20% of all oncological diseases. Notably, primary brain tumors and metastatic CNS lesions are diagnosed with roughly equal frequency in adults.

Current understanding of the Etiology of most nervous system tumors aligns with universal concepts regarding THE ORIGIN OF cancers in any other anatomical Location. There is a group of disorders with a confirmed hereditary basis—phacomatoses (neurofibromatosis, tuberous sclerosis, von Hippel-Lindau disease). A number of tumors are of dysembriogenetic origin (craniopharyngiomas, dermoid and epidermoid cysts, teratomas, etc.). At the same time, brain tumors possess several specific characteristics that distinguish them from neoplasms in other locations.

   ■ Both malignant and benign brain tumors can lead to irreversible nervous system dysfunction and pose a serious threat to the patient's life due to their development within the confined space of the cranial cavity.

   ■ Brain tumors present with neurological disorders and typically lack the characteristic somatic signs of Cancer (such as weight loss, regional lymphadenopathy, elevated ESR, and signs of intoxication, among others).

   ■ The Development of brain tumors is practically never accompanied by metastasis outside the cranial cavity, whereas tumors of other Organs quite frequently metastasize to The Skull and brain.

There is a rather complex and extensive histological Classification of brain tumors. It takes into account the Water/144.html">Origin of the tumor growth, malignancy grade, growth rate, and pattern, which often dictate treatment options and the choice of management strategy. From a practical standpoint, brain tumors can be divided into intra-axial and extra-axial. Dysembriogenetic tumors occupy an intermediate position. Metastatic tumors constitute a separate group.

Intra-axial tumors include those originating from brain tissue Cells. These are primarily astrocytomas, oligodendrogliomas, ependymomas, glioblastomas, as well as tumors arising from embryonic Cell lines: medulloblastomas, neuroblastomas, ependymoblastomas, and several others. During their growth, these tumors destroy and replace normal brain tissue. Extra-axial tumors develop from the Meninges, Cranial Nerves, or, for example, the Pituitary Gland. This group includes meningiomas, schwannomas of the cranial nerve roots (III, V, VIII), and pituitary adenomas. As they grow, such tumors compress the brain and are typically clearly demarcated from the brain tissue. It is important to bear in mind that brain damage can also occur when tumors from the Paranasal Sinuses, scalp, nasopharynx, and other adjacent structures invade the cranial cavity (extracerebral tumors).

Mechanisms of Tumor-Related CNS Damage

Tumor growth triggers Primary and secondary, local and generalized CNS damage. Malignant tumors are characterized by rapid infiltrative growth. Metastasis of malignant brain tumors may occur within the cranial cavity via the CEREBROSPINAL FLUID (CSF) pathways, but distant metastases to other organs outside The Nervous System do not occur. Benign tumors are typically characterized by slow growth accompanied by compression of adjacent brain structures. Thus, primary local Changes in the tumor growth zone boil down to the destruction or compression of brain tissue areas, which naturally leads to the appearance of focal neurological symptoms, encompassing both deficit and irritation symptoms. Edema of the surrounding brain tissue and Impaired Blood supply typically develop around the tumor growth zone (secondary damage). Occasionally, hemorrhages or ischemic events occur within the tumor tissue itself, which may serve as the initial clinical manifestations of the disease and mimic a stroke.

The accumulation of additional tumor tissue volume and the development of surrounding local brain edema within the enclosed space of the cranial cavity eventually lead to increased intracranial pressure at a certain stage of the pathological process (the so-called "mass effect"). Since intracranial pressure is determined by the interaction and balance of three mediums (brain tissue, blood, and CSF), and the intracranial space cannot expand, an increase in the volume of one component can only occur at the expense of another—most commonly at the expense of venous blood and cerebrospinal fluid. At a certain point in the pathological process, compensatory reserves are exhausted, resulting in a significant rise in intracranial pressure, which manifests as a well-known symptom complex. The blockage of CSF Circulation pathways and venous outflow can cause pressure to rise above the constriction point, leading to ventricular enlargement, known as obstructive Hydrocephalus. Sometimes these disorders can present as obstructive attacks with a rapid surge of CSF pressure within the cerebral ventricles and, consequently, intracranial Hypertension. Due to compromised CSF circulation associated with posterior fossa tumors, an abnormal HEAD posture may be observed.

The expanding volume of the neoplasm, combined with elevated intracranial pressure, causes a shift in the topography of brain structures within the cranial cavity (displacement of various brain regions) and the development of herniation syndromes (dislocation disorders). The most frequent variants include the following: in hemispheric mass lesions, the downward Displacement of the medial temporal lobe typically leads to compression of the upper Brainstem at the tentorial notch. In posterior fossa tumors, downward displacement of contents can cause compression of the Medulla Oblongata at the foramen magnum due to cerebellar tonsillar herniation. Compression of the brainstem leads to life-threatening impairment in The regulation of vital organs and systems—primarily causing respiratory and cardiac arrest, which in most cases is the direct cause of patient mortality.

Clinical Manifestations of Brain Tumors

Taking into account the aforementioned pathogenetic mechanisms of tumor-related brain damage, we distinguish: 1) symptoms of focal or local brain damage; 2) generalized (general cerebral) manifestations; 3) herniation (dislocation) symptoms.

Symptoms of focal brain damage are determined by the tumor's location and arise As a result of both the Direct impact of the tumor process (infiltration, tissue necrosis, edema, compression) and secondary damage mechanisms (such as impaired blood supply). These include deficit symptoms such as paresis and paralysis, sensory conduction disorders, ataxia, reduced visual acuity and visual field defects, bulbar symptoms, and other cranial nerve dysfunctions. At the same time, irritation phenomena may also be observed—most commonly partial, secondary generalized, or generalized epileptic seizures, the focal component of which points to the localization of the pathological process. Irritation of the dura mater can cause localized headaches corresponding to the PROJECTION OF THE tumor. Irritation of the floor of the Fourth ventricle by the tumor can provoke intractable vomiting.

Generalized symptoms are primarily caused by the development of intracranial hypertension syndrome. One of the key signs is headache. A systematic, diffuse, and intense cephalgia is characteristic. It is more pronounced in the morning hours, likely due to impaired venous outflow in the supine position during Sleep. Another hallmark manifestation of intracranial hypertension is systematic nausea and vomiting, which typically have a characteristic "central" (brain-origin) quality. They appear at the peak of the headache and are unrelated to prior epigastric discomfort or other signs of gastrointestinal dysfunction.

The progression of intracranial hypertension may be accompanied by altered states of consciousness and certain psychiatric disorders. Patients may develop lethargy and somnolence, apathy toward the surroundings, memory impairment, inappropriate behavior, etc. Elevated intracranial pressure can promote the onset of epileptic seizures. Intracranial hypertension may also be accompanied by transient blurring of Vision. Later, if intracranial hypertension persists, decreased visual acuity and sometimes even blindness typically develop (due to secondary optic atrophy). Fundoscopic examination frequently reveals papilledema (choked discs). Obstructive attacks are characterized by worsening headaches, intractable vomiting, rapidly progressive depression of consciousness, epileptic seizures, and impaired Regulation of Respiratory and cardiac Functions. Death may occur during an obstructive attack. The appearance of signs of herniation at any level indicates the development of a critical phase of the disease.

Depending on the location and specific clinical manifestations, several groups of brain tumors can be identified:

1. Cerebral hemisphere tumors (supratentorial)

   ■ extra-axial

   ■ intra-axial

   ■ intraventricular

2. Sellar and parasellar tumors (chiasmal-sellar region)

3. Posterior cranial fossa tumors (subtentorial)

4. Metastatic tumors

5. Skull Bone tumors

Clinical manifestations of hemispheric tumors

Frontal lobe tumors. The most frequent clinical manifestations of local frontal lobe damage include headache, motor, speech, coordination, sensory olfactory, visual, and psychiatric disorders.

   ■ Paresis of the facial, Tongue, and limb Muscles on the contralateral side of the body.

   ■ Partial (Jacksonian), secondary generalized, and regular generalized epileptic seizures, as well as adversive seizures (with head and eye deviation to the opposite side).

   ■ Motor aphasia (more common in left-sided lesions).

   ■ Frontal ataxia (astasia, abasia, etc.).

   ■ Extrapiramidal disorders (hypokinesia, grasp Reflexes, forced laughter and crying).

   ■ Pseudobulbar syndrome (signs of oral automatism, etc.).

   ■ Decreased visual acuity (Foster Kennedy syndrome).

   ■ Impairment or loss of SENSE OF SMELL.

   ■ The «frontal psyche» symptom complex: euphoria, facetiousness, reduced critical insight into one's condition, propensity for juvenile or flat jokes, impaired Memory and Attention, apathy, untidiness, and other behavioral disorders.

Temporal lobe tumors. Clinical manifestations are relatively sparse. Characteristic features include headaches, temporal lobe Epilepsy attacks, sensory disorders (visual, vestibular, olfactory, gustatory), as well as motor and speech impairments.

   ■ Epileptic seizures (simple or complex partial with secondary generalization), typically accompanied by an aura manifesting as auditory, gustatory, or olfactory hallucinations; psychiatric, epigastric, and other autonomic-visceral disorders; oroalimentary, vocalization, and motor automatisms; psychomotor paroxysms, etc.

   ■ Vestibular disorders.

   ■ Alterations in taste and smell.

   ■ Sensory or amnestic aphasia (more commonly observed in left hemisphere lesions).

   ■ Homonymous hemianopsia.

   ■ Paresis of the contralateral limb muscles.

   ■ Cranial nerve oculomotor lesions (ptosis and pupillary dilation).

Parietal lobe tumors. Characterized by sensory disorders and disturbances of complex spatial functions.

   ■ Sensory disturbances on the contralateral side of the body.

   ■ Partial (Jacksonian sensory) and secondary generalized epileptic seizures.

   ■ Impairments in spatial orientation and body scheme (autotopagnosia).

   ■ Impairments in complex forms of sensory perception (stereognosis, localization).

   ■ Impairment in performing habitual complex sequential movements (apraxia) in lesions of the left hemisphere.

Occipital lobe tumors. Tumors in this localization are typically characterized by various visual disturbances.

   ■ Visual field defects (contralateral homonymous hemianopsia, quadrantanopsia, scotomas).

   ■ Epileptic seizures with a visual aura.

   ■ Phenomena of visual cortex destruction and irritation (visual hallucinations, visual agnosia, metamorphopsia).

Tumors of the Third ventricle and pineal region. These are characterized by impaired cerebrospinal fluid circulation (elevated intracranial pressure, hydrocephalus, occlusive episodes), hypothalamic disorders, and oculomotor impairments.

Certain variants of hemispheric tumors

Meningiomas are extra-axial, slow-growing tumors that are well demarcated from brain tissue. They develop from meningeal cells and account for approximately 15-20% of all primary brain tumors. They typically manifest between the ages of 35 and 55 and occur twice as frequently in women. Meningiomas often arise along venous sinuses and are associated with the dura mater and cranial bones. A distinction is made between parasagittal meningiomas (originating from the sagittal sinus and falx cerebri) and basal meningiomas (in the region of the olfactory groove, sphenoid wings, tuberculum sellae, etc.). Meningiomas can reach large sizes. The initial clinical manifestations of the tumor are generally focal epileptic seizures. Subsequently, symptoms of neurological deficit gradually emerge. The majority of meningiomas are benign tumors that can be successfully removed surgically. In cases of incomplete tumor resection, recurrences may occur after several years.

Gliomas are intra-axial tumors of varying degrees of malignancy, including astrocytomas, oligodendrogliomas (mostly benign), ependymomas (benign), and glioblastomas (malignant). These tumors often grow diffusely or infiltratively without clear boundaries. Some forms contain cysts (astrocytomas) or areas of calcification (oligodendrogliomas), and feature a network of pathological newly formed vessels, necrotic foci, and hemorrhages (glioblastomas). These tumors most commonly manifest with focal epileptic seizures and increased intracranial pressure syndrome, though a rapid development of distinct focal neurological deficits is also possible. The Scope of Surgical treatment depends on many factors, including growth characteristics, topography, feasibility of a safe surgical approach, and the involvement of major Blood Vessels and vital centers. In some cases, total tumor resection can be achieved. Frequently, however, only partial tumor removal is performed. Endoscopic microsurgical techniques are employed for tumors of the ventricular system. Combined treatment utilizing Radiation therapy and Chemotherapy is also possible.

Tumors of the posterior cranial fossa

This is a large group of tumors comprising those of the Cerebellum, the fourth ventricle, and the brainstem (astrocytomas and hemangioblastomas, ependymomas, medulloblastomas), acoustic schwannomas (vestibular schwannomas of the VIII cranial nerve), and meningiomas. Subtentorial tumors are particularly common in children (accounting for up to 60% of all tumors).

Cerebellar and fourth-ventricular tumors cause ataxia (both static and dynamic) and disturbances in cerebrospinal fluid circulation, leading to elevated intracranial pressure, the development of occlusive hydrocephalus, and brainstem compression syndromes (dislocation). Due to impaired CSF dynamics, an abnormal head posture may be observed. In the event of escalating occlusive symptoms and the threat of brain herniation, ventricular puncture and drainage may be utilized as emergency measures. Lesions affecting other PARTS OF THE brainstem can lead to alternating syndromes and bulbar disorders (manifesting as dysphagia, dysarthria, and dysphonia). Many tumors of the cerebellum, fourth ventricle, and brainstem can be resected, though this requires challenging surgical approaches and microsurgical techniques. Combined treatment modalities are frequently applied.

Vestibular schwannomas of the VIII nerve (accounting for up to 7-10% of intracranial neoplasms) present with Hearing loss, coordination deficits, facial sensory disturbances, and signs of Facial Nerve involvement. Cranial radiography (using specialized projections) reveals an enlargement of the internal acoustic meatus on the side of the tumor. Microsurgical techniques are now widely employed in the removal of VIII nerve schwannomas.

Tumors of the sellar-chiasmatic region

These are neoplastic lesions of the sella turcica and optic chiasm, including pituitary adenomas and craniopharyngiomas (dysontogenetic tumors). Pituitary tumors may be functionally active (hormone-secreting) or non-functioning. Clinically, they are characterized by visual disturbances (decreased visual acuity, visual field defects), impaired cerebrospinal fluid circulation, and hypothalamo-pituitary dysfunctions (manifesting as either hyperfunction or hormone deficiency). In hormone-secreting pituitary adenomas, the latter may be associated with elevated production of prolactin, Growth Hormone, ACTH, etc.

Prolactin-secreting adenomas (prolactinomas) are accompanied by menstrual irregularities, galactorrhea, and other symptoms. Growth hormone-producing adenomas manifest as gigantism in young patients and acromegaly in adults (enlargement of the hands and feet, coarsening of facial features). ACTH-secreting adenomas lead to Cushing's syndrome (hypertension, characteristic truncal fat deposition, hirsutism, etc.). Non-functioning adenomas present with symptoms of panhypopituitarism (obesity, reduced performance capacity, Skin pallor, decreased sexual function, and low blood pressure).

Neuro-ophthalmological evaluation, sellar radiography, CT and MRI, hormone assays, and endocrinological assessment are of paramount importance in the Diagnosis of pituitary adenomas. The growth of prolactin-secreting tumors can be arrested by the administration of dopamine agonists (bromocriptine). Pituitary adenomas can be resected via transnasal-transsphenoidal, frontal, or frontotemporal approaches. The management strategy for patients with pituitary adenomas is largely determined by the severity and progression of visual disturbances.

Craniopharyngiomas are dysontogenetic tumors occurring in children and adolescents, resulting from the incomplete regression of an embryonic pharyngeal pouch diverticulum that participates in The formation of the anterior pituitary gland. They account for 6-9% of all pediatric brain tumors. These tumors are located in the region of the sella turcica and contain calcifications and cysts. Endocrine disorders, visual disturbances, and intracranial hypertension are characteristic. Radical tumor resection is feasible. When necessary (in cases of adrenal insufficiency), steroid hormone replacement is administered in the pre- and postoperative periods. Neuroendocrine disorders (such as diabetes insipidus and adrenocortical insufficiency) either develop or persist following surgery.

Metastatic brain tumors

Brain metastases are detected in 15-20% of patients who succumb to oncological diseases. Within the overall structure of intracranial tumors, metastatic lesions account for approximately 30-50%. The most frequent primary sources of brain metastases are Lung Cancer, breast cancer, and renal cell carcinoma. Although patient history assists in recognizing this pathology, the primary source of metastasis remains unidentified in 15% of all cases. Metastases can be solitary or multiple, affecting both the brain parenchyma and surrounding Tissues (the latter indirectly confirming the metastatic Nature of the lesion). Management of such conditions involves glucocorticoids (dexamethasone), radiation therapy, and less frequently, chemotherapy. Solitary metastases can be removed surgically. The average life expectancy of patients following the diagnosis of untreated brain metastases is approximately 1 month. It is important to bear in mind that the onset of neurological deficits in patients with malignancies of various organs may stem not from metastases, but rather from paraneoplastic neurological syndromes.

Diagnosis of brain tumors and the diagnostic value of instrumental imaging Methods

The challenge in early or timely diagnosis of brain tumors is that when initial warning symptoms appear (e.g., isolated olfactory disturbances, visual field defects, leg weakness, or the first-ever epileptic seizure), most patients are unaware that these disorders originate from the nervous system and require consultation with a neurologist or neurosurgeon. Consequently, the responsibility for properly interpreting a patient's Complaints and symptoms during their initial presentation, as well as directing them along the appropriate diagnostic pathway, frequently falls upon general practitioners. Suspicion of a potential brain tumor during an initial patient visit can be established based on the following signs (criteria):

   ■ Systematic, persistent, generalized headaches

   ■ Persistent localized headaches

   ■ Gradually progressive focal neurological deficits

   ■ Partial, secondary generalized, or generalized epileptic seizures

   ■ Rapidly progressive mental and behavioral changes

Suspicion is heightened by the detection of optic disc edema (papilledema) on funduscopy, a midline shift on echoencephaloscopy, and an elevated protein level in the CSF. Readily accessible diagnostic methods include funduscopy, skull radiography, and echoencephaloscopy.

Funduscopy makes it possible to identify signs of optic disc edema, which confirms increased intracranial pressure, or atrophic changes, potentially of a secondary nature. However, the results of neuro-ophthalmological examination depend on the stage and other parameters of the space-occupying lesion and do not yield definitive results in all cases.

In most cases of brain tumors, skull radiography reveals no abnormalities. Extremely rarely, due to high tumor density, the neoplasm may be visible on standard skull radiographs. In some instances, signs of bone destruction adjacent to the tumor are detected. Nevertheless, craniography is essential for identifying and ruling out Other types of pathology, such as traumatic skull injuries, foreign bodies, Osteomyelitis, postoperative defects, areas of calcification, myeloma, and others. Signs of increased intracranial pressure on skull radiographs (accentuated vascular markings, digital impressions, Osteoporosis of the sella turcica, etc.) do not serve as definitive proof of a space-occupying lesion and require cautious interpretation, as they most frequently indicate prior, potentially perinatal, nervous system injuries.

Echoencephaloscopy frequently allows for the detection of indirect signs of a hemispheric space-occupying lesion in the form of a midline shift in the opposite direction. Exceptions include cases of polar tumor localization and tumors of the posterior cranial fossa, where the diagnostic value of the method is negligible.

CT, MRI, and cerebral angiography are the most informative diagnostic tools. These modern neuroimaging modalities (CT, MRI) help determine the location, density, and STRUCTURE OF THE tumor. They allow clinicians to clarify the tumor growth pattern, the presence and severity of perifocal brain edema, signs of associated ventricular distortion, dislocation syndrome, and secondary vascular changes. Gadolinium-enhanced MRI enables the precise Determination of the border between the tumor and the perifocal edema of the brain tissue. Although MRI offers higher resolution, both CT and MRI provide only a rough approximation of the tumor's histological nature, essentially characterizing alterations in X-ray or magnetic density of the brain tissues.

In cases of brain tumors, cerebral angiography can reveal the tumor's own vascular network or, conversely, the displacement of cerebral vessels and an avascular zone at the site of the suspected neoplasm. Accounting for the specific vascularization Features of the tumor is crucial for planning appropriate surgical tactics, which helps prevent massive Hemorrhage and other vascular complications during surgery. Following the Introduction of CT and MRI, cerebral angiography lost its primary independent role in the diagnosis of intracranial tumors; nevertheless, it remains widely used as an important Complement to these methods.

Cerebrospinal fluid analysis quite often reveals elevated pressure and an increased protein count. Lumbar puncture is contraindicated in the presence of pronounced intracranial hypertension, signs of brain herniation (dislocation), or suspected tumor localization in the posterior cranial fossa, temporal lobe, or cerebral ventricles.

Stereotactic biopsy allows for the precise determination of the tumor's histological structure, enabling the Selection of the most appropriate combined therapy strategy even before surgery. In domestic neurology and neurosurgery, this method currently has limited application.

As a rule, routine Laboratory tests (complete blood count, urinalysis, etc.) reveal no abnormalities in patients with brain tumors. Given the availability of CT or MRI, other Instrumental Diagnostic Methods for brain tumors are of only secondary importance and lack sufficient diagnostic value to formulate a surgical treatment plan.

Depending on the clinical manifestation, the Cytology/practical/136.html">Differential diagnosis OF brain tumors must be performed against other types of space-occupying brain lesions, such as cerebrovascular accidents, abscesses, Various Forms of traumatic brain compression, parasitic diseases, and others.

Principles of Treatment for Patients with Brain Tumors

Surgical methods are the most effective and widespread modalities in the comprehensive, combined treatment of brain tumors. Various forms of radiotherapy and chemotherapy are less efficacious. The optimal surgical technique ensures total tumor resection while avoiding severe brain damage and postoperative neurological deficits. However, in some cases, only partial tumor resection is feasible due to difficult surgical access and the infiltration of tumor tissue into major blood vessels, Dural Venous Sinuses, and vital brain regions. Under such circumstances, tumor recurrence can be expected within a few months or years. Adherence to certain conditions and the application of advanced modern technologies help prevent complications and improve surgical outcomes:

■ Precise three-dimensional visualization of the space-occupying lesion

■ Determination of tumor vascularization characteristics

■ Prior performance of stereotactic biopsy

   ■ Administration of neuroprotective agents and dehydration therapy before and during surgery

   ■ Utilization of ultrasonic aspiration, microsurgical, and endoscopic techniques

■ Early Prevention of postoperative complications

Tumor removal is best performed before the onset of pronounced neurological deficits and decompensation. However, deciding on the necessity of early surgical intervention—when instrumental findings are clear yet neurological symptoms remain minimal and predominantly subjective—presents an immense challenge for both the patient and the surgeon. Such a decision must be discussed taking into account all specific circumstances of the case and the patient's individual psychological profile.

The main modalities of radiotherapy boil down to the implantation of radiation sources directly into the tumor tissue (radiosurgical methods) and external beam radiotherapy. Radiotherapy as a standalone treatment is most commonly used for pituitary adenomas, certain types of gliomas, radiosensitive tumors of the skull base, and other neoplasms that are difficult to access surgically. Radiotherapy is more broadly utilized as part of combined treatment for radiosensitive brain tumors following partial resection (e.g., ependymomas, medulloblastomas). Gamma therapy is the most widely practiced approach in domestic clinical settings. Post-radiation complications may include increased brain edema, delayed radiation necrosis, post-radiation encephalopathy with dementia, alopecia, and others.

The capabilities of chemotherapy in neuro-oncology are limited. It is primarily used as an adjunct to surgical and radiation treatments.

With escalating intracranial hypertension and clinical deterioration during preoperative preparation, pathogenetic dehydrating therapy (furosemide, mannitol, dexamethasone) is administered. In cases of progressive occlusive hydrocephalus and signs of brain dislocation, emergency interventions may include ventricular puncture and drainage, which help decompress the cerebrospinal fluid spaces of the cranial cavity and buy time for surgical decompression and tumor resection. Following the removal of subtentorial tumors, long-term CSF drainage is occasionally required as well. Symptomatic therapy is administered as needed, including analgesics and antiepileptic drugs.

Spinal Cord Tumors

Spinal cord tumors are conventionally subdivided into primary and secondary. The first group comprises tumors originating from the spinal cord itself, its meninges, and blood vessels. The secondary group consists of neoplasms arising from surrounding tissues that invade the spinal canal and damage the spinal cord. Metastatic tumors represent a distinct category of spinal cord neoplastic lesions. Primary tumors are further differentiated into intramedullary (originating from the neural parenchyma) and extramedullary (developing from the nerve roots, meninges, and blood vessels). Extramedullary tumors may be located subdurally (beneath the dura mater) or epidurally (outside the dura mater). Furthermore, spinal cord tumors can be classified according to their anatomical location within the cervical, thoracic, or lumbosacral segments. Special cases include craniovergetral junction tumors and cauda equina tumors. The most frequent extramedullary tumors are meningiomas, neurinomas, hemangioblastomas, and lipomas, whereas astrocytomas and ependymomas predominate among intramedullary tumors.

Developing within the confined space of the spinal canal, spinal cord tumors lead to compression or destruction of the spinal cord and nerve roots, as well as disturbances in blood and CSF circulation, which further exacerbates spinal cord injury.

The clinical manifestations of spinal cord tumors comprise several symptom complexes. These include radicular-meningeal disorders, transverse spinal cord lesion syndrome (encompassing segmental and long-tract spinal symptoms), and impaired patency of the CSF pathways.

Radicular pain is particularly characteristic of extramedullary tumors. It tends to worsen at night and in the recumbent position, can be unilateral or bilateral, and is frequently girdle-like in character. The pain is exacerbated by pressure or Percussion over the spinous processes at the level of the tumor. Hyperesthesia and paresthesias frequently appear within the zone of radicular innervation.

Destruction of the posterior and lateral horns of the spinal cord by a tumor results in a pattern of segmental dissociated sensory disturbances and autonomic dysfunction corresponding to the site of the pathological process, which closely mimics syringomyelia and therefore necessitates differential diagnosis. Involvement of the anterior horn motor Neurons manifests as peripheral paresis of the muscles innervated by the affected segments. Destruction of motor and sensory fibers within the anterior, lateral, and posterior columns of the spinal cord leads to long-tract motor and sensory deficits localized below the level of the lesion. Motor impairments typically present as lower spastic paraparesis. The Nature of sensory disturbances depends on the tumor's location. An extramedullary tumor causes ascending sensory deficits, which originate in the feet and spread upward to the level of the spinal cord lesion. In contrast, with an intramedullary tumor, sensory disturbances initially appear at the level of the lesion and subsequently spread downward. At various Stages of the tumor process, one may observe partial transverse spinal cord involvement (including hemisection — Brown-Séquard syndrome) or complete transverse involvement. All these alterations may be bilaterally symmetrical or asymmetrical. In the majority of cases, spinal cord tumors eventually cause bowel and bladder dysfunction. Depending on the tumor's location, these can be of central or peripheral type. At all stages of spinal cord tumor involvement, autonomic and trophic changes are evident in the affected limbs, and their severity generally increases with the progression of the motor deficit. The boundaries of sensory and motor impairments are determined by the level of the spinal cord lesion and the degree of transverse involvement.

Extramedullary tumors are characterized by the early onset of radicular symptoms during the Initial Stages of the disease. Progressive spinal cord compression leads to long-tract motor and sensory disturbances, which initially manifest in the feet and gradually ascend to the level of the affected segments. Symptoms of segmental apparatus involvement at the level of the tumor may also occur. Extramedullary localizations are typically marked by early alterations in the CSF and blockade of the spinal subarachnoid space.

For intramedullary tumors, the absence of radicular symptoms in the early stages is considered typical; however, the initial signs of the disease include segmental sensory disturbances of the dissociated type. Owing to the involvement of the anterior horns of the spinal cord, peripheral paresis may also develop. Subsequently, signs of transverse spinal cord involvement emerge, accompanied by severe motor and sensory long-tract deficits. As noted previously, hypesthesia in these cases spreads downward from the level of the affected segments. Subarachnoid space blockade develops much later with such tumors.

The progressive intensification of radicular or segmental disorders, along with the appearance of spinal long-tract signs, should always raise suspicion of a primary or secondary spinal cord tumor. Spinal radiography is the simplest diagnostic method that can detect evidence of metastatic disease, rule out other verttebrogenic causes of spinal cord dysfunction, and reveal indirect signs of a mass lesion (destruction or osteoporosis of vertebral arches, enlargement of intervertebral foramina, etc.). CSF analysis in spinal cord tumors typically reveals elevated protein levels. Impairment of subarachnoid space patency can be detected using special dynamic tests (Queckenstedt, Stookey, and Poussepp tests). However, MRI and myelography remain the most informative diagnostic modalities for confirming suspected spinal cord tumors and precisely localizing the process.

Spinal cord tumors must be differentiated from syringomyelia, AMYOTROPHIC LATERAL SCLEROSIS, multiple sclerosis, vertebrogenic radiculomyeloischemia, and a range of degenerative spinal cord disorders.

Surgical resection is the most effective treatment for spinal cord tumors. These tumors are usually approached posteriorly via decompressive laminectomy. Most intradural extramedullary tumors can be removed radically. Advanced microsurgical techniques and ultrasonic aspiration also enable the successful removal of numerous intra- and intratracheal/extramedullary tumors (such as ependymomas). Certain glial tumors (astrocytomas, oligodendrogliomas) can only be resected partially, in which case spinal cord compression is alleviated by cyst drainage. In some cases, palliative Procedures are performed, such as decompressive laminectomy to reduce spinal cord compression. Surgical treatment for many benign spinal cord tumors offers the prospect of significant clinical improvement, even in the presence of paraplegia and when performed in elderly patients.

Clinical Problems

Case 1

A 32-year-old man, while at work in his usual state of health, suddenly experienced a generalized seizure with loss of consciousness, convulsions, tongue biting, and frothing at the Mouth, lasting approximately 4 minutes. According to witnesses, tonic followed by clonic seizures began in the right arm and leg and subsequently spread to the entire body. Postictally, the patient exhibited lethargy, weakness in the right arm, and speech difficulty, which gradually improved over the course of an hour and resolved completely. For the past 4–6 months, he has been troubled by headaches, predominantly in the frontoparietal region, which have recently become more intense and regular. The headaches tend to appear toward the evening, occasionally at night or in the morning, and may be accompanied by nausea. Neurological examination reveals hyperactive deep tendon reflexes on the right side. No other abnormalities are detected. Fundoscopic examination shows retinal venous engorgement and slight blurring of the optic disc margins. Echoencephalography demonstrates a 4 mm shift of the M-echo from left to right.

   • How would you characterize the patient's neurological status at the time of examination?

   • List the primary neurological deficits.

   • How should the seizure accompanied by loss of consciousness and convulsions be classified?

   • Explain the postictal lethargy, speech difficulty, and right-arm weakness.

   • Determine the most likely type of cephalgic syndrome.

   • What do the fundoscopic findings indicate?

   • Explain the results of the echoencephalography.

   • Name the main signs of elevated intracranial pressure.

   • Formulate the topographic diagnosis.

   • Formulate and substantiate a provisional clinical diagnosis.

   • Name other conditions that could account for the reported symptoms.

   • What additional anamnestic details should be specifically targeted and clarified?

   • Outline the protocol for instrumental neurological examination.

   • What is the clinical rationale for performing a head CT or MRI?

   • Outline the patient management plan.

   • Describe the required general somatic examination.

   • Specify the therapeutic interventions required at this stage.

   • What factors may trigger clinical decompensation in this patient?

   • List potential neurological complications in the event of an unfavorable disease course.

   • Explain the Pathophysiology of brain herniation syndromes and list their primary anatomical types.

Option 2

A 48-year-old patient has been experiencing intermittent frontal and occipital headaches for 3 months, accompanied by unsteadiness and gait ataxia. At the onset of the disease, headaches occurred at any time of day following physical exertion, overheating, or fatigue; recently, however, they have become nearly constant, most severe in the morning, and are frequently accompanied by nausea and occasionally vomiting. When changing head position, the patient occasionally notes transient blurring of vision. Difficulty swallowing has developed. The condition is progressively worsening. Neurological examination reveals coarse bilateral horizontal nystagmus, dysphagia, dysphonia, and depressed pharyngeal reflexes. Muscle hypotonia is present. Coordination tests are performed with moderate bilateral ataxia. The patient is unable to stand in the Romberg position and falls. Maintaining balance in a sitting position is difficult. Echoencephaloscopy revealed no shift of the M-echo.

   • How would you characterize the patient's neurological status at the time of examination?

   • List the primary neurological deficits.

   • How can the episodes of visual disturbance be classified?

   • How can the swallowing difficulties be explained?

   • Characterize the headache syndrome.

   • Characterize the motor function impairment.

   • Explain the results of the echoencephaloscopy.

   • Formulate the topographic diagnosis.

   • Formulate and justify a provisional clinical diagnosis.

   • Outline the key areas for differential diagnosis.

   • What additional anamnestic details should be specifically targeted and clarified?

   • Outline the plan and objectives for instrumental neurological evaluation.

   • Describe the protocol for the somatic examination.

   • Outline the list of therapeutic interventions required at this stage.

   • List the MAIN TYPES OF ataxia.

   • Name other potential causes of gait disturbance.

✵ State the primary signs of increased intracranial pressure.

   • Enumerate the possible neurological complications in the event of an unfavorable disease course.

   • Determine the prognosis of the disease.

Option 3

A 50-year-old patient reports a gradual decrease in visual acuity, coarsening of facial features, and enlargement of the Nose, supraorbital ridges, feet, and hands over the past 2–3 years. They are troubled by frequent frontotemporal headaches. Upon examination, noteworthy findings include an enlarged nose, prominent supraorbital ridges, a massive Mandible, and large hands and feet. Pupils are symmetric; direct and consensual light reflexes are sluggish. Bitemporal hemianopia is present.

   • List the main neurological disorders.

   • Explain the changes in facial features, as well as the shape and size of the hands and feet.

   • How can the visual acuity impairments be explained?

   • How can the visual field defects be interpreted?

   • Determine the level of lesion within the visual system.

   • Explain The Nature and origin of the cephalgic syndrome.

✵ Formulate the topical diagnosis.

   • Formulate and substantiate the preliminary clinical diagnosis.

   • Outline the main directions for differential diagnosis.

   • What additional anamnestic details, supplementing the provided history, need to be specifically clarified?

   • Outline the plan and objectives for instrumental neurological examination.

   • Describe the skull X-ray findings characteristic of this disease.

   • What somatic examination should be performed?

   • Outline the list of therapeutic interventions required at this stage.

   • Enumerate the possible neurological complications in the event of an unfavorable disease course.

   • Name the most common hormonally active pituitary adenomas.

   • Describe the possible treatment options for pituitary tumors

  • Determine the prognosis of the disease.

Option 4

A 68-year-old woman noticed discomfort and clumsiness in her feet when walking a year ago. She had a long-standing history of vertebral lumbosacral radiculitis. Gradually, due to progressive weakness and muscle tension in her legs, she began to walk with difficulty, supporting herself on surrounding objects. Concurrently, tingling sensations and dulling of sensation in her legs spread upward to the level of the knee joints, and subsequently to the abdomen. About a month ago, intermittent Urinary Incontinence developed. A neurological examination revealed a significant decrease in leg strength, accompanied by brisk tendon reflexes and pathological Babinski signs. A reduction in pain and Temperature Sensation was determined below the level of T₁₁. Spine radiographs showed signs of widespread osteochondrosis and deforming spondyloarthritis. Descending myelography revealed a contrast block at the level of T₁₀.

  • List the main neurological disorders.

  • Describe the motor function impairment syndrome.

  • Describe the sensory disturbance syndrome.

  • What is the Diagnostic significance of the progression of sensory disorders?

  • How should the results of spondylography be interpreted?

  • Formulate indications for descending myelography.

  • Explain the myelography results.

  • Formulate and justify the topical diagnosis.

  • Formulate a preliminary clinical diagnosis.

  • Outline the main directions for differential diagnosis.

  • Outline the scheme for instrumental neurological examination.

  • Describe the necessary somatic evaluation.

  • What other neurological syndromes are typically observed in this disease?

  • Describe the cerebrospinal fluid changes characteristic of this case.

  • What abnormalities may be detected during cerebrospinal fluid hydrodynamic tests?

  • What additional anamnestic details, supplementing the presented information, need to be specifically clarified?

  • List the most common causes of gait and lower limb movement disorders.

  • Describe the principles and capabilities of surgical treatment.

  • Determine the prognosis of the disease.

Option 5

A 65-year-old man suffering from lower back and leg pain had repeatedly received treatment for vertebral radiculitis. Within 24 hours following some physical exertion, he developed weakness in his legs and experienced difficulty walking. Shortly thereafter, urinary incontinence appeared. A neurological examination revealed decreased leg strength, increased muscle tone and tendon reflexes, and pathological plantar signs. Sensory impairment affecting pain and temperature was detected starting from the level of L₁ on the left. Approximately 5 years ago, Prostatic Adenoma was diagnosed. Spinal radiographs revealed areas of destruction in the vertebral bodies and arches of T_11-12 and L₂, as well as in the pelvic bones. Descending myelography demonstrated a contrast block at the level of T₁₁.

  • List the main neurological disorders.

   • Describe the motor function impairment syndrome.

   • Describe the sensory impairment syndrome.

   • Explain the results of spondylography.

   • Determine the indications for descending myelography.

   • Explain the results of the myelographic examination.

   • Formulate and justify the topical diagnosis.

   • Formulate a preliminary clinical diagnosis.

   • Outline the main directions for differential diagnosis.

   • Describe the protocol for instrumental neurological examination.

   • What is the rationale for CT or MRI examination?

   • Indicate the necessary methods for somatic examination.

   • Describe the cerebrospinal fluid changes characteristic of this condition.

   • What abnormalities can be detected during cerebrospinal fluid hydrodynamic tests?

   • What additional anamnestic details should be specifically clarified beyond what has already been presented?

   • List the most common causes of impaired gait and lower limb mobility.

   • Outline the patient management plan.

   • Describe the principles and options of surgical treatment.

   • Determine the prognosis of the disease.

Option 6

A 38-year-old woman complains of weakness and sensory disturbances in her legs, as well as difficulty urinating. Walking is accompanied by a sensation of stiffness and tension in the leg muscles. About four months ago, she developed itching in the left hemithorax, followed by tingling and decreased skin sensation in a band-like distribution just above the costal margin. Over time, the sensory disturbances became bilateral. Gradually, clumsiness and weakness developed in the legs, more pronounced on the right side. She began to experience mild pain in the lower thoracic spine. Physical examination reveals a significant decrease in lower limb muscle strength, increased muscle tone, and gait difficulty. Knee and Achilles reflexes are brisk, with bilateral pathological Babinski reflexes. Hypesthesia is present from the level of the costal margin downwards across the trunk and lower extremities. There is impaired muscle-joint sense in the feet.

   • List the primary neurological disorders.

   • Explain the nature and origin of the sensory disturbances.

   • What is the diagnostic Significance of the progression of sensory disorders?

   • Describe the motor deficit syndrome.

   • Formulate the topical diagnosis.

   • Formulate and justify a provisional clinical diagnosis.

   • Outline the main directions for differential diagnosis.

   • Outline the plan for instrumental and laboratory investigations.

   • Indicate the required methods for somatic examination.

   • What additional anamnestic details, beyond those already provided, should be specifically clarified?

   • Describe the cerebrospinal fluid findings typical of this condition.

   • What abnormalities may be detected during CSF pressure dynamics tests?

   • Formulate the indications for myelography.

   • What is the rationale for CT or MRI examination?

   • Outline the patient management plan.

   • Describe the principles and potential options for surgical treatment.

   • Determine the prognosis of the disease.