Metropolitan Neurology

Neurological Conditions

Migraine

Migraines typically cause moderate or severe head pain. They are often felt on one side of the head (the name is a shortening of the Greek word ‘hemicrania’, meaning ‘half-head’), but they can occur anywhere in the head, often sitting in the forehead or at the base of the skull. They may have a throbbing or pulsating character. They are often preceded by, or associated with pain in the neck, and more rarely elsewhere in the body.
Migraines are ‘headaches plus’, in that the pain is typoically associated with one or more other features. These may include nausea, vomiting, sensitivity to lights, noises or smells. They are often made worse by any form of movement. Migraine headaches usually last hours, or even a few days. They can be shorter in children.

About one-quarter of people with migraine experience aura – a period of neurological dysfunction, usually affecting vision, that typically precedes the headache phase. More rarely aura causes numbness and tingling down one side of the body, dizziness, an inability to speak properly, or transient weakness.

About one in five people with migraine will get a warning that an attack is coming. This prodrome, or premonitory phase may include symptoms such as fatigue, yawning, craving for sweet or salty foods, euphoria, or frequent urination. At least as many people will experience a postdrome, or migraine hangover, during which, even though the pain has largely settled, they do not feel back to their usual selves, often struggling to concentrate or exert themselves physically.

Tension-type headache

Most people experience tension-type headaches at some point in their lives. This is a mild ache, sometimes feeling like a band around the head, not usually associated with any other features, that will go by itself, or with a simple painkiller such as paracetamol or ibuprofen. Most people can carry on their usual activities while they have a tension-type headache. They rarely cause any problems unless they become frequent or chronic.

Cluster headache

Cluster headache is a rare but characteristic headache in which people experience invariably unilateral pain, in and around the eye, associated with activation of nerves over which we do not have voluntary control (autonomic nerves). This causes problems such as redness and watering of the eye on the side of the pain, running or blockage of the nose, swelling around the eye, drooping of the eyelid, excessive sweating, and agitation or restlessness. People with cluster headache find it very difficult to stay still. They may rock to and from, or pace around the house. In extreme cases they may actually hit their head against a wall to try to distract themselves from the pain.

Cluster headaches often wake people in the night, sometimes so reproducibly that the condition has been dubbed the ‘alarm clock headache’. Cluster attacks tend to be shorter than migraines, usually lasting no more than a couple of hours; but people can have multiple cluster attacks in one day. Cluster headaches often come in bouts lasting  a few weeks. There is a clear tendency for these bouts to come at certain times of year, often when day length changes in the spring or autumn

Idiopathic intracranial hypertension

The human brain and spinal cord are surrounded, nourished and supported by cerebrospinal fluid (CSF). When the pressure of the CSF rises or falls, this can cause headaches. The headache of elevated CSF pressure is typically worse when lying down, often waking people in the morning; it can be accompanied by double vision, pulsatile tinnitus, and transient loss of vision on bending over or standing up. This can be due to a structural lesion within the brain, such as a brain tumour or a blood clot in the cerebral veins, but if there is no serious problem of that type, then the diagnosis is likely to be idiopathic intracranial hypertension (IIH).

IIH is more common in women (often young women) than men, and characteristically (though not invariably) affects people who are overweight. In such people, weight loss is the mainstay of treatment. Otherwise, medication can be helpful, but in some cases neurosurgical interventions are required. It is important to treat IIH proactively to ensure preservation of visual function.

Low CSF volume headache

The commonest cause of a low CSF volume headache is a junior (now called resident) doctor. Specifically, it is the consequence of a prolonged leak of CSF after a lumbar puncture (or spinal tap). In those cases, people experience headaches that are typically worse on standing, and better when they lie down. Post-lumbar puncture headaches are usually self-limiting, but there can be other causes for CSF leaks (often related to spinal trauma), and they can happen spontaneously; in those cases symptoms can persist much longer and can be trickier to diagnose and treat.

Low CSF volume headaches will often respond to bed rest, hydration, caffeine and painkillers. If they do not, epidural blood patches (placing about 30 mL of the patient’s own blood over the leak to plug it) can in many cases solve the problem.

Trigeminal neuralgia

Trigeminal neuralgia is characterised by unilateral brief, electric shock-like facial pains, most often in the distribution of the second and third branches of the trigeminal nerve (the cheek and jaw, respectively). Some patients have a dull background pain in between attacks. Attacks can be triggered washing, shaving, talking, brushing teeth, or the cold. There is typically a refractory period after an episode during which the triggering stimuli do not bring on an immediate attack.

Trigeminal neuralgia is a condition that predominantly effect older people; it is rare in those <55 years. It is often characterised by relapses and remissions; at least 50% of patients have breaks in attacks of at least six months. If associated with ipsilateral facial spasm it is known as tic doloureux. MRI imaging may show compression or contact of the trigeminal nerve root by a blood vessel, or more rarely inflammation of the area where the trigeminal nerve enters the brainstem.

Epilepsy

Epilepsy is simply “a condition in which the sufferer is prone to recurrent epileptic seizures”. Practically, epilepsy is diagnosed when two  or more seizures have occurred. The lifetime risk of epilepsy is 3-5%.

Seizures are due to abnormal, excessive synchronized discharge of cerebral neurons. The effect of this process depends on whether the discharges are focal or affect the whole of the brain at once. The most common focal seizures arise from the temporal lobes, causing rising epigastric aura, déjà vu, fear, and automatisms such as lip smacking or picking at things. Frontal lobe seizures can cause head turning. Parietal lobe seizures can cause focal twitching (the so-called Jacksonian march) or pins and needles that spread over seconds. Occipital lobe seizures are very rare, manifesting as elemental blobs of colour in the visual fields.

Focal seizures may spread to become generalised, or seizures may arise in a generalised fashion de novo. Generalized seizures, if transient, can simply cause brief absence spells; if more prolonged, they can cause convulsions, incontinence, and tongue-biting. Very rarely, people can come to harm or even die during seizures. Most people with epilepsy respond well to anti-seizure medication, though perhaps one-third of people need to take more than one medication to achieve this.

Parkinson’s disease

Parkinson’s disease is a movement disorder characterised by progressive, typically asymmetric slowness of movement (bradykinesia), stiffness (rigidity), and a tremor at rest. Many sufferers also experience loss of their postural reflexes, leading to unsteadiness and falls. Some patients with Parkinson’s also develop non-motor symptoms, which can include apathy, anxiety, excessive salivation, vivid dreams, constipation, and memory problems.

The condition was first described by the surgeon-apothecary (proto-GP) James Parkinson in his Essay on the Shaking Palsy in 1817. In the 1960s it was discovered that the basic problem in Parkinson’s disease is loss of brain cells that contain the neurotransmitter dopamine. People lose 50% of their dopamine-containing cells before Parkinson’s disease becomes apparent.

Treatments for Parkinson’s either replace dopamine (in the form of levodopa, which the brain can convert into dopamine), stop dopamine being broken down so quickly, or stimulate dopamine receptors directly. Most people will respond well to treatment, though over a number of years various motor complications such as freezing, or excessive muscle activity, can start to appear.

Essential tremor

Very few people are able to hold their outstretched arms absolutely still. Most of us have a mild physiological tremor, which is usually worse if we are tired, stressed, or have taken certain medications (such as asthma inhalers). Some people experience an exaggerated tremor of this type, absent at rest but problematic when trying to use the hands, as they get older. Benign, or essential tremor of this type often runs in families. It is only rarely so severe as to adversely impact people’s quality of life. It often responds well to a small dose of beta-blockers.

Stroke

Strokes are caused by a sudden interruption of blood supply to an area of the brain, either because of a blood clot or bleeding. Exactly what effect this has depends on which blood vessel is blocked or bleeds, what area of the brain is affected, and what that bit of the brain does. Blood clots may form in the heart because of an irregular heart rhythm, or in the blood vessels of the neck or the brain itself because of damage from smoking, diabetes, high blood pressure, and/or high cholesterol levels. Blood vessels may bleed because of the long-term effect of high blood pressure, or more rarely because of inflammation or genetic defects in the connective tissues that make up the vessels themselves.

The commonest effects of strokes are weakness and numbness affecting the face and arm on one side, or the whole side of the body. Depending on whether the stroke affects the dominant side of the brain, speech may also be affected. More rarely strokes can cause sudden loss of vision, dizziness and vertigo, unsteadiness or inco-ordination. Whatever the effects are, the onset is sudden, and the symptoms are generally at their worst almost immediately.

Strokes caused by blood clots can be treated with clot-busting treatments (thrombolysis) or by physical removal of the clot (thrombectomy). The NHS hyperacute stroke units specialize in such treatments, and anyone suspected of having a stroke should be assessed in such a unit as quickly as possible: time is brain! Outpatient assessment focusses on understanding the causes of stroke, and on optimizing measures to prevent strokes from happening again, or in the first place. A vital part of this is recognizing when people may have had a transient ischaemic attack (TIA or ‘mini-stroke’) as urgent treatment in such cases can significantly lower the risk of a full-blown stroke occurring thereafter.

Transient ischaemic attacks (TIAs)

The signs, symptoms, and underlying causes of TIAs are exactly the same as those of strokes, except that in the case of TIAs, everything recovers back to normal within 24 hours (in fact, usually within minutes). As mentioned above, urgent assessment and treatment is required in cases of TIA, as this can significantly reduce the chances of the patient going on to have a much more devastating event within a few days. All patients considered to have had a probable TIA should have a full cardiovascular and neurological assessment, imaging of the brain and main blood vessels that supply the brain, and cardiac investigations if appropriate.

Myopathy/myositis

Muscle weakness is unusual, but can occur as a result of inflammation of the muscles (myositis), which is often quite painful, or of more gradual impairment of muscle function (myopathy), which can be due to a number of causes, including metabolic disturbances (such as vitamin D deficiency), endocrine disorders (such as an underactive thyroid gland), or genetic conditions. A thorough clinical assessment, appropriate blood tests, electrical tests of muscle function and (very occasionally) a muscle biopsy will usually provide a diagnosis, and treatment if possible.

Neuropathy

Nerves can go wrong in a few different ways. The commonest cause of a peripheral neuropathy (that is, generalized dysfunction of the nerves in arms and legs, rather than in the brain or spinal cord) is axonal degeneration, of which the commonest causes in the UK are diabetes, alcohol, lack of vitamin B12 and/or folic acid. Less commonly nerves can become inflamed, sometimes acutely in aftermath of an infection, as in Guillain-Barré syndrome, or more chronically in cases of systemic inflammation, or where there are abnormal (para)proteins in the blood. Generalized neuropathies usually affect the longest nerves in the body first, hence symptoms typically start in the toes or the fingers, before spreading up the arms and legs in the so-called ‘glove and stocking’ pattern.

Other Conditions

Details to follow shortly on:

Carpal tunnel syndrome

Ulnar neuropathy

Myasthenia gravis

Motor neuron disease

Multiple sclerosis

Cervical disc disease

Lumbar disc disease

Vestibulopathy

Insomnia

Alzeheimer’s disease

Vascular dementia

Lewy body disease

Frontotemporal dementia