Neurology embodies some of the most dreadful diseases known to man. Every neurological disorder is disheartening, each characterised by unique frustrations for patients and their families. It is difficult to quantify the distress and misery these afflictions impose on their victims, and even harder to appreciate the despair and anguish they evoke in those who care for them. These diseases come with unimaginable physical and psychological burdens, and crushing demands on human and material resources. They impose either a debilitating morbidity, or a hasty mortality.
It is clearly hard to compare the impact of different neurological diseases. Some neurological disorders however stand out because of the consternation their names evoke, and the terror that follows in their wake. The nervous system ailments in the list below pose exacting therapeutic challenges, demonstrating recalcitrant resistance to all attempts at treatment or cure. This list sets out to emphasise the urgency for neuroscience to find a remedy for each of them, but it does not intend to belittle the horror of the disorders omitted from it. The choice of the number 13 is, sadly, self-evident. Here then are 13 exceptionally dreadful neurological disorders.
Peripheral neuropathy is ubiquitous in the neurology clinic, and it is broadly classified into axonal and demyelinating. Neuropathy may result from reversible situations such as overindulgence in alcohol, uncontrolled diabetes, or Vitamin B12 deficiency. Neuropathy is often just a minor inconvenience when it manifests with sensory symptoms such as tingling and numbness. It may however be debilitating when it presents as limb paralysis, or complicated by major skeletal deformities. At the severe end of the spectrum of neuropathy are the hereditary forms such as Charcot Marie Tooth disease (CMT) and familial amyloid polyneuropathy (FAP).
Ataxia, in lay terms, is incoordination. This typically manifests as an unsteady gait and clumsiness. Ataxia converts all activities of daily living into burdensome chores. Whilst many types of ataxia are preventable or reversible, primary ataxias are progressive and carry a dismal outlook. In this category are Friedreich’s ataxia, ataxia telangiectasia, and the 43 spinocerebellar ataxias (SCA).
Dystonia marks its presence by distressing movements and painful postures. At its most benign, dystonia is only a flicker of the eyelid – blepharospasm, or a twitch of one side of the face – hemifacial spasm. At the extreme end, it produces continuous twisting and swirling motions, often defying all treatments. The causes of dystonia are legion, but the 14 primary dystonias stand out by their hereditary transmission and marked severity.
Dementia is often the scourge of longevity. Its name strikes terror because it insidiously colonises the brain cells that make us who we are. The most prominent dementia is Alzheimer’s disease, but it has equally dreadful companions such as frontotemporal dementia (FTD), dementia with Lewy bodies (DLB), and progressive supranuclear palsy (PSP).
Brain cancer hardly needs any introduction. It is either primary, arising from the brain cells, or metastatic, spreading to the brain from other organs. Some primary brain cancers, such as meningiomas and pituitary adenomas, are relatively treatable. Many others are unfortunately ominously malignant, and the most dreadful in this category is surely the spine-chilling glioblastoma multiforme.
Multiple sclerosis (MS)
Multiple sclerosis is a very common disease, and gets more common the further away you get from the equator. It is the subject of intense research because of the devastation it foists on predominantly young people. Many drugs now ameliorate, and even seem to halt the progression of relapsing remitting MS (RRMS). This is however not the case with primary progressive MS (PPMS) which, until the introduction of Ocrelizumab, defied all treatments. There are many modifiable and non-modifiable risk factors contending for the elusive title of the cause of MS, and the real reason why nerves in the central nervous system inexplicably start to loose their myelin sheaths remains a puzzle.
Muscular dystrophy is an umbrella term that covers a diverse range of inherited muscle diseases. The most devastating, on account of its early onset and unrelenting progression, is Duchenne muscular dystrophy (DMD). Adult neurologists will however be more familiar with later onset muscular dystrophies such as myotonic dystrophy type 1 and facioscapulohumeral muscular dystrophy (FSHD). The malevolent onset and pernicious progression of muscular dystrophies justifies their inclusion in the pantheon of dreadful neurological disorders.
Tetanus is an eminently preventable disease, now almost wiped out in developed countries by simple immunisation. It however continues its pillage and plunder in the developing world. It strikes young and old alike, often invading the body through innocuous wounds. The culprits in tetanus are tetanospasmin and tetanolysin – the deadly toxins of the bacterium Clostridium tetani. The disease is classified as generalised, localised, cephalic, or neonatal tetanus. It is characterised by painful spasms which manifest as lockjaw (trismus), facial contortions (risus sardonicus), trunkal rigidity (opisthotonus), and vocal cord spasms (laryngospasm). The disease is awfully distressing and, when advanced, untreatable. It is a stain on the world that this avoidable disorder continuous to threaten a large number of its inhabitants.
Rabies, a rhabdovirus, is a zoonosis; it is transmitted to man by a wide range of animals such as dogs, bats, racoons, and skunks. It is the quintessential deadly neurological disease, as horrific as its depiction by Steven King in his book and film, Cujo. Rabies manifests either as an encephalitic (furious) or a paralytic (dumb) form. It wreaks havoc by causing irritability, hydrophobia (fear of water), excessive sweating, distressing priapism, altered consciousness, and almost uniform fatality. Whilst there are vaccines to protect against rabies, a cure has eluded neuroscientists.
Creutzfeldt Jakob disease (CJD)
CJD is the most iconic of the prion diseases: these disorders are as horrendous as they are enigmatic, defying categorisation as either infections or neurodegenerative diseases. More puzzling is their ability to be either hereditary and acquired. CJD exists in the classic or variant form, but both share an obstinately lethal course, and a uniformly mortal ending.
Huntington’s disease (HD)
Huntington’s disease is an iconic eponymous neurological disorder which is marked by the vicious triumvirate of chorea, dementia, and a positive family history. It is an awful condition, often driving its victims to suicide. It is a so-called trinucleotide repeat expansion disorder which exhibits, amongst other things, genetic anticipation – successive generations of affected families manifesting the disease at an earlier age, and with more severe features.
Motor neurone disease (MND)
Also known as amyotrophic lateral sclerosis (ALS), MND is simply devastating. Recognising no anatomical boundaries, it is an indiscriminate scavenger of both the central and peripheral nervous systems. It creeps up on the neurones and evokes early muscle twitching (fasciculations) and cramps. It then gradually devours the nerves resulting in muscle wasting, loss of speech, ineffectual breathing, and impaired swallowing. It is undoubtedly one of the most dreadful diseases to ever hound the human body.
Spinal cord injury
Nothing is quite as heart-wrenching as the sudden loss of body function that results from spinal cord injury. This may cause paralysis of both legs, paraplegia, or all four limbs, quadriplegia. This life-changing disorder is often accompanied by loss of control over bowel and bladder functions, and complications such as bed sores and painful spasms. The impact of spinal cord injury, whatever the cause, is simply devastating and it clearly marks the condition as dreadful.
No list ever does justice to the items left out of it. As for all lists, this will surely be subject to debate, or perhaps some healthy controversy. So please leave a comment. And to raise the spirits, the next blog post has the more uplifting title of 7 eminently curable neurological disorders!