NEUROLOGY AND NEUROSURGERY - YU. V. ALEKSEEENKO - 2014

HEADACHES

Headaches are among the most frequent Complaints encountered by neurologists and general practitioners. According to the modern Classification of the International Headache Society (IHS), headaches are categorized into primary (where no organic cause is identified and the pain syndrome constitutes the primary condition), secondary (caused by organic lesions of the Central Nervous system, other Organs, or systemic disorders), and cranial neuralgias and facial pains.

Primary headaches include migraine, tension-type headache, cluster headache, and several other rare types. Secondary headaches may result from HEAD or neck trauma, vascular disorders, other intracranial pathological processes, exposure to various substances, infections, or Homeostasis disorders. Additionally, this category includes headaches and facial pains caused by conditions affecting the Skull, neck, eyes, Nose, ears, sinuses, and the masticatory system. Finally, there are headaches associated with psychiatric disorders, while cranial nerve neuralgias form a distinct group.

A critical challenge during a patient's initial presentation with headache complaints is ruling out a secondary Etiology that would necessitate urgent diagnostic and therapeutic interventions. However, in general medical practice, such situations occur much less frequently than specialists often assume. Warning signs that demand caution include the sudden onset of severe headache (Subarachnoid Hemorrhage?), a headache progressively worsening over several weeks or months, altered consciousness, or epileptic seizures (Brain tumor?); alongside the headache, the presence of persistent focal neurological deficits or meningeal signs (stroke, meningitis, vasculitis?).

Migraine

Migraine is one of the most prevalent forms of primary headache. It manifests as periodically recurring headache attacks, often pulsating in nature and typically localized to one side of the head (hemicrania). In most cases, it is accompanied by nausea, sometimes vomiting, photophobia, phonophobia, and a complex of autonomic-vascular symptoms. Migraines affect 10-15% of the adult population, occurring 1.5-2 times more frequently in women than in men. The prevalence peaks around the age of 40 in women and 35 in men. Various Forms of migraine are distinguished: migraine without aura, migraine with aura, childhood periodic syndromes that may precede or accompany migraine, retinal migraine, basilar-type migraine, and other variants.

The exact causes of migraine remain incompletely understood, though a genetic predisposition to the condition is evident. An increased sensitivity (sensitization) of the Brainstem trigeminovascular system plays a major role in triggering migraine attacks, driven by an imbalance in neurotransmitter and neuroendocrine-humoral mechanisms. The Development of a migraine attack involves a characteristic sequence of vascular responses (vasoconstriction followed by vasodilation) and local reversible ischemia in areas of the Cerebral Cortex. Recently, The Role of nitric oxide in the mechanisms of attack generation has been actively investigated. This condition can be classified as a psychosomatic disorder rooted in a disruption of the natural biorhythmological regulation of psychophysiological processes.

The primary manifestation of migraine is periodically recurring headache attacks. A migraine attack can occur at any time of day, frequently beginning during nighttime Sleep, in the early morning, or immediately upon waking. The frequency of attacks ranges from a few in a lifetime to several per week, with the majority of patients experiencing 1-2 attacks per month.

Quite often, the onset of a migraine attack is associated with various triggering factors. These include dietary irregularities, particularly prolonged intervals between meals. Attacks can also be provoked by the consumption of specific foods (cocoa, chocolate, cheese, citrus fruits, alcohol, coffee, certain food additives, and preservatives, among others). Well-known triggers include disrupted sleep duration and schedules, as well as negative emotions. A wide range of environmental factors can precipitate a migraine attack, such as changes in barometric pressure and intense rhythmic sensory stimulation. Other recognized risk factors include hormonal fluctuations during menstruation or ovulation, The Use of oral contraceptives, exacerbations of concurrent medical conditions, and the administration of vasodilators. However, it should be noted that in most patients, exposure to triggers does not invariably lead to an attack. It is hypothesized that individuals with migraines develop an increased susceptibility to triggering mechanisms, characterized by heightened perception of environmental stimuli, internal state changes, and sometimes intense cravings for specific foods.

The progression of a migraine attack comprises the prodromal phase, the aura, the headache phase itself, resolution, and the postdromal phase. Prodromal symptoms develop several hours before the attack and are observed in approximately 40% of patients. Most commonly, these include mood changes, irritability, alterations in psychomotor reactions and intellectual performance, and unexplained malaise—such as lethargy, fatigue, and drowsiness. An aura—transient symptoms of cerebral cortex irritation resulting from the initial humoral-vascular mechanisms of the attack—occurs in 10-15% of patients, though it does not necessarily precede every attack. Occasionally, a migraine attack may terminate at the aura stage. Visual aura is the most frequent manifestation, presenting as photopsias, scotomas, hemianopia, shimmering jagged lines, streaks, spots, sparks, blurriness, or distorted perception of the size and shape of objects ("Alice in Wonderland" syndrome). In some cases, paresthesias develop, spreading from the fingertips to the entire upper extremity, face, Lips, and Tongue. Motor impairments such as hemiparesis and transient speech disorders may also occur.

The headache gradually intensifies from a feeling of discomfort to severe pain over the course of 0.5-1 hour. The duration of the attack is typically 12-24 hours. The headache is usually unilateral, though it may affect both sides of the head. It often recurs on the same side of the head, but alternating sides are also observed. In approximately 50% of cases, the headache has a throbbing quality. Concurrently, patients experience intolerance to bright light, noise, and odors, accompanied by nausea, vomiting, and anorexia. Sensitivity of the Neck Muscles and scalp may also develop. Any physical activity consistently exacerbates the pain, and drowsiness ensues. Interestingly, sleep can bring a migraine attack to an end.

Simultaneously with the cephalic syndrome, signs of pronounced autonomic dysfunction develop: chills, fever, malaise, diaphoresis, palpitations, dry Mouth, a sense of suffocation, yawning, epigastric discomfort, frequent polyuria, etc. The intensity and diversity of these manifestations are such that this state can unhesitatingly be interpreted as a mixed autonomic crisis. It should be emphasized that a migraine attack is essentially the most prominent variant of autonomic paroxysms, featuring characteristic generalized and specific local manifestations in the form of the migrainous headache syndrome.

In more than 50% of patients, the termination of a migraine attack occurs following sleep. In some cases, particularly in children, the attack ceases after vomiting. The postdromal period is characterized by weakness, feeling washed out, irritability, impaired concentration, and Muscle soreness, lasting anywhere from 1 hour to several days.

Discussing the main variants of migraine, It is important to note that alongside the classic migraine with typical aura, there are cases where a typical aura is followed by a different type of headache (such as tension-type headache), as well as variants where a typical aura occurs without any headache at all.

Formalized diagnostic criteria exist for both migraine without aura and migraine with aura.

Migraine without aura

   A. At least 5 attacks fulfilling criteria B–D

   B. Headache attacks lasting 4–72 hours (untreated or unsuccessfully treated)

   C. Headache has at least 2 of the following characteristics:

   • unilateral Location

   • pulsating quality

   • moderate or severe pain intensity (inhibiting routine activities)

   • aggravation by routine physical activity

D. During the headache, at least one of the following symptoms occurs:

   • nausea and/or vomiting

   • photophobia and phonophobia

   E. Presence of at least one of the following conditions:

   • medical history, general somatic and neurological examinations do not confirm the secondary Nature of the pain syndrome or the presence of any other disorder

   • medical history, general somatic and neurological examinations suggest a disorder, but it is ruled out by targeted diagnostic investigations

   • such a disorder is present, but the onset of migraine attacks is not temporally related to it

Migraine with aura

   A. At least 2 attacks fulfilling criterion B

   B. Headache accompanied by at least three of the following four characteristics:

   • one or more fully reversible aura symptoms indicating focal cerebral cortical and/or brainstem dysfunction

   • at least one aura symptom develops gradually over more than 4 minutes, or 2 or more symptoms occur in succession

   • aura symptoms do not last longer than 60 minutes; if more than one aura symptom is present, the total duration increases proportionally

   • headache follows the aura within a certain interval not exceeding 60 minutes (it may also begin concurrently with or before the aura)

   C. Presence of at least one of the following conditions:

   • medical history, general somatic and neurological examinations do not confirm the secondary nature of the pain syndrome or the presence of any other disorder

   • medical history, general somatic and neurological examinations suggest a disorder, but it is ruled out by targeted diagnostic investigations

   • such a disorder is present, but the onset of migraine attacks is not temporally related to it

In patients with migraine, brain MRI typically reveals no evidence of structural CNS lesions. Nevertheless, such imaging is necessary if there are abnormalities on neurological examination, if a typical migraine attack first occurs after the age of 40, if the frequency or intensity of migraine attacks progressively increases, if the characteristics of migraine-associated symptoms change, or if new psychiatric symptoms emerge in connection with the onset of migraine attacks.

When discussing the Therapeutic management of migraine, it is essential to distinguish between the acute Treatment of migraine attacks proper and the prophylaxis of migraine attacks. For the acute treatment of migraine attacks, widely used agents include 1) simple and combination analgesics, 2) NSAIDs, and 3) disease-specific medications. To evaluate the efficacy of a given agent in treating a migraine attack, a set of specific criteria has been proposed:

   • absence of pain at 2 hours;

   • reduction of headache from moderate or severe intensity to mild or complete absence of pain at 2 hours;

   • consistent efficacy in two out of three attacks;

   • no recurrence of headache and no need for rescue medication within 24 hours following successful treatment.

Among simple and combination analgesics, the most popular are acetylsalicylic acid (500–1000 mg), paracetamol (500 mg), Citramon, Ascofen, Sedalgin, metamizole, Sedalgin-Neo, Pentalgin, etc. A crucial condition for the effective use of these and other medications is oral administration as early as possible at the very onset of pain. This is because gastrointestinal dysregulation (gastrostasis), which develops during a migraine attack, impairs the normal absorption of drugs. These disturbances can be overcome by adding a prokinetic agent—10 mg of metoclopramide (G. Votans, 1974). Nonsteroidal anti-inflammatory drugs can be successfully used to treat migraine attacks—diclofenac 50–100 mg (including in the form of rectal suppositories), ibuprofen, nimesulide, etc. NSAIDs can and sometimes must be combined with metoclopramide. At the same time, potential gastrointestinal side effects, the development of agranulocytosis, arterial hypotension, etc., must be kept in mind. To resolve medication-overuse headache, the intake of medications should be restricted—to 15 days per month for simple analgesics and 10 days per month for combination analgesics.

Specific medications are also used to treat acute migraine attacks. The first group comprises preparations containing ergot Alkaloids (e.g., ergotamine tartrate 0.1%, 15–20 drops or 1–2 mg in tablets). Recently, their use has significantly declined due to a high probability of side effects and the availability of safer, highly effective pharmacotherapeutic alternatives.

The second group of specific-action agents consists of triptans. Triptans represent an entirely new Class of drugs that act as 5-HT1B/1D serotonin receptor agonists. The primary Mechanisms of action of triptans in migraine involve the constriction of dilated cranial extracerebral vessels, the limitation of neuropeptide release and plasma protein extravasation across the meningeal vessels, and the suppression of pain signal propagation along the trigeminovascular system. Below are the most well-known triptan medications:

   • Sumatriptan - 25, 50, 100 mg (oral formulations, including fast-release forms), 25 mg suppositories, 10, 20 mg nasal spray, 6 mg subcutaneous injection;

   • Zolmitriptan - 2.5, 5 mg (oral formulations, including orally disintegrating tablets), 2.5, 5 mg nasal spray;

   • Naratriptan - 2.5 mg (oral formulations);

   • Rizatriptan - 10 mg (oral formulations, including orally disintegrating wafers);

   • Almotriptan - 12.5 mg (oral formulations);

   • Eletriptan - 20, 40 mg (oral formulations);

   • Frovatriptan - 2.5 mg (oral formulations).

It should be emphasized that these medications are not universal analgesics. They should not be used for the treatment of othercephalgic syndromes, with the exception of cluster headache. They are effective in approximately 60% of patients who do not respond to NSAIDs and are effective when taken at any point during an attack. However, the earlier such a drug is taken, the better its efficacy. The optimal time for administering triptans is the very onset of the headache. Triptans should not be taken during the aura phase. The use of triptans should ideally be limited to 10 days per month. It is known that subcutaneous sumatriptan is effective within 2 hours in 80% of patients. Some triptans may be effective when other members of this group fail. A certain degree of Variability in response is observed—efficacy in only two out of three attacks should be considered a good outcome. Triptans exhibit a dose-dependent effect accompanied by an increased frequency of side effects.

With most agents, headache recurrence is possible—a phenomenon where cephalgia intensifies following a pain-free period or presents as a mild headache while taking the medication within a 24-hour window. This issue occurs more frequently with the use of triptans and NSAIDs. Approximately 15–40% of patients taking oral triptans report pain recurrence. A second dose of triptans is effective in the majority of cases. If the first dose of triptans proves ineffective, taking a second dose is useless.

The most frequent side effects of triptans include chest discomfort, nausea, distal paresthesias, and fatigue, among others. The use of triptans is contraindicated in untreated arterial Hypertension, coronary artery disease, cerebrovascular diseases (history of stroke), Raynaud's syndrome, Pregnancy and Lactation, age under 18 (with the exception of the nasal spray), age over 65, and severe hepatic or renal impairment.

The high frequency and prolonged duration of migraine attacks, which significantly disrupt patients' education, professional activities, and daily functioning, along with comorbid disorders and the risk of complications, are indications for preventive treatment. Lifestyle changes, activity modification, and the elimination of trigger factors are recommended. The core concept and direction of therapeutic and preventive measures in migraine is the restoration of the natural mechanisms of biorhythmological regulation of psychophysiological processes in the broadest sense. The decision regarding pharmacological prophylaxis should be made thoughtfully and jointly by the physician and the patient, with a full understanding of all the complexities involved in such a program and the necessary effort required from the patient. Preventive treatment involves the use of beta-blockers, antidepressants, calcium channel blockers, antiepileptic drugs, vasoactive agents, and NSAIDs. Patients are also advised to keep a headache diary.

Tension-type headache

Tension-type headache is the most common form of primary headache, manifesting as cephalgic episodes lasting from several minutes to several days. The pain is typically bilateral, pressing or tightening in nature, of mild to moderate intensity, does not worsen with routine physical activity, and is not accompanied by nausea, although photophobia or phonophobia may occasionally occur. The pain usually appears shortly after waking up and persists throughout the day, fluctuating in intensity. It is typically alleviated by positive emotions and psychological relaxation. Pain exacerbation often occurs against the Background of emotional distress. Along with cephalgia, such patients complain of pain, tension, or a feeling of tightness in the occipital region, the back of the neck, and the shoulder girdle. Some patients exhibit increased sensitivity or even tenderness of the pericranial muscles.

Patients with tension-type headache quite frequently complain of heightened anxiety, depressed mood, apathy, irritability, and poor nighttime sleep quality. Such manifestations of anxiety or depressive disorders are very characteristic and range from mild to severe. Trigger factors for tension-type headache include emotional stress and static muscle tension associated with prolonged forced positioning of the neck and head during work, driving, etc. Tension-type headaches are classified as episodic (occurring no more than 15 days per month) or chronic (occurring more than 15 days per month). The transformation of episodic tension-type headache into the chronic form can be driven by several factors. Among psychological factors, chronic stress situations, depressive disorders, and anxiety states are the most dangerous. Overuse of symptomatic painkillers is also a major factor contributing to chronification. Pericranial muscle dysfunction and the maintenance of muscle tension facilitate the transition of tension-type headaches into a chronic form. In some patients, a combination of migraine and tension-type headache is observed.

Tension-type headache is largely a psychogenic disorder. Its development is primarily attributed to the heightened sensitivity (sensitization) of nociceptive structures and the functional insufficiency of descending inhibitory pathways in the brainstem. However, many of the underlying mechanisms of this process remain unclear.

Treatment of tension-type headache primarily involves the Prevention and correction of emotional, anxiety-depressive, hypochondriacal, and somatoform disorders. It is essential to identify, prevent, and treat tension in the pericranial, neck, and shoulder girdle muscles. A crucial objective is also the prevention of medication overuse, i.e., the development of medication-overuse headache. All of these measures reduce the likelihood of tension-type headache transforming into a chronic form.

Patients are advised to adopt lifestyle changes, optimize their workload, and eliminate triggering factors. Simple and combination analgesics as well as NSAIDs are used episodically. Course treatment with muscle relaxants (tolperisone, tizanidine) is advisable. In some cases, the prescription of antidepressants may be required (amitriptyline, serotonin reuptake inhibitors such as paroxetine, fluoxetine, sertraline, etc., and serotonin-norepinephrine reuptake inhibitors such as duloxetine, etc.). Beta-blockers and calcium channel blockers are used for prophylactic purposes. Non-pharmacological Methods are recommended: relaxation and behavioral therapy, biofeedback, acupuncture, massage, and manual therapy.

Clinical Cases

Case 1

A 24-year-old female has been suffering from headache attacks since the age of 10, occurring 3–4 times a month. The onset of the cephalgic attack is usually preceded by a deterioration in mood and a feeling of depression. Sometimes the onset of an attack can be linked to physical and emotional stress, or sleep deprivation. Typically, against this background, blurred contours of objects and a flickering zigzag line appear in the right visual field. This is followed by the onset and gradual intensification of a bursting pain in the left temple and Eyeball, which subsequently becomes throbbing in nature. Photophobia, intolerance to bright light, noise, and odors develop, and speaking becomes difficult. Examination during the attack reveals facial pallor and dark circles under the eyes. Blood pressure is 115/80 mm Hg, pulse is 72 beats/min, regular. Attacks occur at various times of the day, last for several hours, and usually terminate with nausea and vomiting. Taking paracetamol or metamizole provides minimal relief. If the patient manages to fall asleep, the headache usually subsides. No focal neurological deficits are detected. The interictal period is frequently characterized by pulse lability, a tendency toward tachycardia upon minimal exertion, orthostatic or clinostatic reactions manifested as palpitations, head noise, and dizziness, a sensation of tension and breathing difficulty, and weather sensitivity. Skull X-rays reveal no abnormalities. The patient's mother has a long history of paroxysmal headaches that typically precede menstruation.

   • List the leading neurological disorders.

   • How should the visual disturbances preceding the headache be interpreted?

   • What stages can be distinguished in the Development of the attacks?

   • What signs indicate the migrainous nature of the headaches?

   • Formulate a preliminary clinical Diagnosis.

   • Outline the main directions for Differential diagnosis.

   • Specify the necessary instrumental examination methods and their purpose.

   • Name the main Clinical forms of migraine.

✵ What characteristics of cephalalgia can be suspected in the patient's mother?

   • List the factors that trigger or promote attacks.

   • What is status migrainosus?

   • Describe the methods for aborting migraine attacks.

   • Outline the management strategy and core components of interval (interictal) therapy.

   • Determine the prognosis of the disease.

   • Formulate preventive recommendations.

Infectious and inflammatory disorders of The Nervous System are extremely diverse and can be classified according to the localization and nature of the inflammatory process, the type of pathogen, and other criteria. However, this group of pathological conditions shares one defining characteristic: nervous system involvement resulting from the Direct impact of infectious agents.

Among the main clinical variants, traditionally distinguished are meningitis (infectious and inflammatory involvement of the nervous system with primary affection of the pia mater) and encephalitis (with primary affection of the brain parenchyma). It should be noted that such a division is rather conventional, since isolated involvement of the Meninges, as well as the brain parenchyma without affecting adjacent structures, is virtually impossible. Less common are infectious and inflammatory lesions of the Spinal Cord, its meninges, spinal nerve roots, peripheral nerves, and spinal ganglia (myelitis, epiduritis, radiculitis, ganglionitis, etc.). Etiologically, nervous system infections are classified into bacterial, viral, fungal, and protozoan.