Mens Sana in Corpore Sano - Disorders of the Brain Quiz

Answer: Batten Disease

First identified in 1903 by the British paediatrician Frederick Batten, this disease is a member of a family of disorders referred to as NCLs or Neuronal Ceroid Lipofuscinoses. Batten Disease, a very rare genetic condition which affects just three births in every one hundred thousand, manifests itself during childhood often at around the age of two although the onset may be delayed depending on the variety of the disease which afflicts the sufferer; this may be infantile, late infantile, juvenile or adult forms.

Although many of the genes that cause this distressing illness have been identified, sadly there is currently no cure for Batten disease and the condition is terminal.

Answer: Epilepsy

This affliction has been recognised since at least 400 BCE with sufferers often viewed with suspicion and treated as if they were victims of demonic possession. Great advances in understanding this condition were made during the 1860s by the neurologist John Hughlings Jackson who catalogued many types of episodes.

The term 'Jacksonian March' is used to describe the progression of tremors which gradually move through the body often resulting in unconsciousness. It was once normal practice to prevent the victim swallowing their tongue by putting fingers in the mouth.

As a nine year old, I recall a teacher at my school almost losing her little finger completely after putting it in the mouth of a young girl who was experiencing a 'grand mal' seizure during morning assembly! Fortunately with modern understanding, medication and changes to the individual's lifestyle, many of those with epilepsy are able to lead full and perfectly normal lives.

Answer: Stroke

A stroke is defined as any incidence of bleeding within the brain. Stroke can be a devastating affliction and many television films and advertisements have been produced in order to raise awareness of the condition in recent years; the mnemonic F.A.S.T. cannot have escaped the notice of many in the United Kingdom! A stroke is caused by a blood clot in the brain which prevents the circulatory system from providing this organ with the food and oxygen that it requires leading to rapid cell death. The clot may have formed within the brain or may have formed elsewhere, been dislodged and travelled there in the blood stream. Fortunately, the very nature of the brain means that it can recover and 'rewire' itself but only on the proviso that medical assistance is readily available, ideally within a window of two or three hours.

In many of these cases, a near full recovery can be made. Unfortunately some bleeds are so massive and so rapid that death from stroke can be almost immediate.

Answer: Creutzfeldt-Jakob Disease (CJD)

It was after the discovery of the prion, or proteinaceous infectious particle, in 1982 by Stanley B. Prusiner that an entire family of diseases was identified and became known collectively as spongiform encephalopathy. Universally fatal, Creutzfeldt-Jakob disease is particularly devastating and with symptoms including dementia, hallucinations and changes in personality, it is not unusual for this disease to claim the life of its victims within a single year.

The affliction was first described during the 1920s by German neurologists Hans Gerhard Creutzfeldt and Alfons Maria Jakob and it is rare; the disease occurs in around just 6500 people globally.

The role of prions in the human body is still little understood. They occur in everyone, the harmless form of the prion known as PrP, covering the outer surfaces of the neurones in the brain.

In the development of CJD it is believed that the changes which cause this disease may be triggered either genetically or through the introduction of the mutated prion from outside the body.

It was this fact which caused such concern during the 1990s when Bovine Spongiform Encephalopathy or BSE came to the fore after the revelation that material from infected livestock had been introduced into the human food chain. Frighteningly, prions are incredibly resilient and it is this property that makes the agent highly dangerous as, unlike other agents, these proteins are entirely unaffected by the heat of cooking.

Answer: Kuru

The word 'Kuru' in the language of the Fore tribe of Papua New Guinea means 'trembling', the involuntary tremors most noticeable in the early stages with fits of uncontrollable laughter only appearing in the latter stages of the disease. Kuru was first described in 1957 by doctor Vincent Zigas and scientist Daniel Gajdusek, who discovered that Kuru was unique to the Fore tribe; instances of the disease were unheard of immediately outside their tribal boundary.

After death it was cultural practice for the women and children of this tribe to prepare and eat the internal organs, especially the brains, of any dead relatives.

The disease only affected the women and children as adult males were forbidden from taking part in these ritualistic funeral meals.

As a result of their research into Kuru, the Indonesian government banned the practice of cannibalism and the disease virtually disappeared. Gajdusek was awarded the 1976 Nobel Prize for Medicine for his work in the field. It was only with the later discovery of prions that the cause of Kuru could be attributed to these agents.

Answer: Huntington's Disease (HD)

Doctor George Huntington was just 22 years old when, in 1872, he published a paper describing this disease which now bears his name. Huntington's Disease, or 'Chorea' as it was once known, is a rare genetic disorder which affects both males and females and which occurs in between five and eight people in one hundred thousand per head of population. Chorea is a word from the Greek language meaning 'dance' and which describes the jerks and twitches that the body is subjected to by this condition.

The symptoms of Huntington's disease usually appear between the ages of 25 and 55, the disease progressing slowly and taking between ten and twenty five years for sufferers to succumb to its effects. It is not known why Barranquitas should have such a high incidence of the affliction but research has shown that over half of the population of twenty-five thousand carry the gene that causes the disease.

In 1993 research finally isolated the gene responsible for the disease and the protein which this gene produces which was later named Huntingtin. Tragically, because this genetic disease often manifests itself during later life, it is impossible to know whether or not the disease might have been passed on to any offspring.

It is useful for those that think they may be carriers of HD to check this through genetic testing.

Answer: Cerebral Palsy

As a condition, Cerebral Palsy is the result of something going wrong during the brain's development. This may have occurred either within the womb due to infection, complications or trauma during the birthing process, or at some stage relatively shortly afterwards.

Historically, it was believed that the condition was caused by oxygen deprivation during pregnancy and, although this may be the case in some rare instances, these cases have been proved to be very much in the minority. The first descriptions in any detail of a number of conditions that we now refer to as Cerebral Palsy were made by the surgeon William Little in 1860. Because this condition remains unchanged during the entire lifetime of those afflicted, never showing any sign of improvement or deterioration, Cerebral Palsy is not considered to be an illness.

The effects of CP include restricted bodily movement due to a 'tightening' of the muscles, sight and speech difficulties and learning difficulty although many of those with this condition do have normal mental faculties.

The incidence of CP is at around one in every five hundred births but dependent on the care that they receive and the severity of their disability, it is not uncommon for CP sufferers to live a relatively long life.

Answer: Wernicke-Korsakoff Syndrome

This syndrome is one of the few neurological illnesses that can be attributed self-inflicted alcohol abuse. Named for doctors Carl Wernicke and Sergei Korsakoff, the syndrome that bears their names is caused by a total dependence on alcohol which, although high in calories contains no nutrients at all.

In serious cases the sufferer is severely malnourished and the lack of Vitamin B1 or Thiamine is the ultimate cause of the development of this syndrome. The illness is indicated by involuntary eye movements, eventual partial or total paralysis of the eyes, memory loss and a lack of balance and co-ordination whilst standing and walking with hallucinations manifesting as the illness progresses. Treatment for Wernicke-Korsakoff can be relatively successful but with a seriously malnourished sufferer it is important that the vitamin deficiency is corrected before work on the effects of malnourishment takes place.

The major areas of the brain that are affected by this set of symptoms are the mammilliary bodies and the hippocampus, and in the later stages of the illness sufferers of Wernicke-Korsakoff syndrome often invent their own imaginary worlds to compensate for the loss of memory that the syndrome has afflicted them with.

Answer: Bipolar Disorder

Clinical depression, commonly referred to as unipolar disorder, is caused by a chemical imbalance within the brain leading to sufferers experiencing thoughts of their own death, general worthlessness, poor concentration and disturbed sleep patterns. Those suffering with bipolar disorder experience these symptoms during one phase of their illness and an intense feeling of euphoria in another phase. Moving between periods of the deepest depression to feelings of immense creativity and self esteem within a very short time frame, it is believed that many great artists, musicians and writers may have suffered from episodes of bipolar illness. Treatment for the condition varies between the use of chemical compounds, such as lithium salts, which are highly effective in stabilising mood, therapy and counselling or a combination of any or all of these.

Therapies, such as Cognitive Behavioural Therapy (CBT), help those affected with bipolar disorder by examining situations in which they may become overwhelmed and breaking them down into smaller situations that they might find easier to deal with.

Answer: Attention Deficit Hyperactivity Disorder

Since the introduction of the term Attention Deficit Disorder in the early 1980s, this condition is, with between three and seven per cent of all mental health referrals for children, the most common. The condition affects both boys and girls, usually under the age of eight years old, although it appears to affect more boys to girls in a ratio of around three to one.

It used to be believed that ADHD as it is more often referred to, was simply an excuse for nothing more than bad behaviour. Those children affected by this condition show signs of intense agitation, an inability to concentrate and bad-temper, all of which can lead to difficulties in every aspect of relationships with their families, at school and amongst their peer group. It has been observed that ADHD appears to have a tendency to run in families and as a result scientists have been searching for a genetic element to the condition with research demonstrating that a lack of the chemical dopamine in the brain is a significant and contributory factor. ADHD is usually treated with the stimulant methylphenidate, otherwise known by its trade name of Ritalin.

It's surprising that a condition such as this should be treated with a stimulant!