The 13 most dreadful neurological disorders…and the groups standing up to them

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.

Brain Art. Ars Electronica on Flikr. https://www.flickr.com/photos/arselectronica/7773544158

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. 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.

Neural pathways in the brain. NICHD on Flikr. https://www.flickr.com/photos/nichd/16672073333

The nervous system ailments in the list below pose exacting therapeutic challenges, resistant as they are 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 the top 13 most dreadful neurological disorders…all with gold links to the associations helping to defeat them.

Working Brain. Gontzal García del Caño on Flikr. https://www.flickr.com/photos/euskalanato/2052487054

Ataxia

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 Spinocerebellar ataxia (SCA)Friedreich’s ataxia, and Ataxia telangiectasia. You may read more about ataxia in these previous blog posts: The 43 spinocerebellar ataxias: the complete checklistsOld drugs, new roles?, and Will Riluzole really be good for cerebellar ataxia?

Brain tumours

Brain cancers hardly need any description. They are 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 tumours, are, relatively, treatable. Many others are unfortunately ominously malignant. The most dreadful in this category is surely the spine-chilling glioblastoma multiforme. You may check out these previous blog posts for more on these tumuors: Calming the rage of brain tumours: hope for a dreaded cancerMaggots, viruses and lasers: some innovations for brain tumoursand Are steroids detrimental to survival in brain tumours?

Peripheral neuropathy

Peripheral neuropathy is ubiquitous in the neurology clinic. 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. Read more in these blog posts: The 52 variants of CMT… and their practical checklistsWhat’s looming at the frontline of peripheral neuropathy? and Will a pill really hold the cure for CMT?

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 a relentlessly rapid course, and a uniformly fatal end. You may read more in these previous blog posts titled Final day of ANA 2015- Prions center stage, and What are the links between Prion diseases and Parkinsonian disorders?

Dementia

Dementia is the scourge of longevity. Its name strikes terror because it insidiously colonises the 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) and Dementia with Lewy bodies (DLB). Read more on dementia in these blog posts: How bright is the future for Alzheimer’s disease?Alzheimer’s disease: a few curious things, and Alzheimers disease and its promising links with diabetes.

Dystonia

Dystonia marks its presence by distressing movements and painful postures. At its most benign, dystonia is only a twitch of the eyelid (blepharospasm) or a flicker 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 primary dystonias stand out by their hereditary transmission and marked severity. Read more on dystonia in these blog posts: Why does dystonia fascinate and challenge neurology? and Making sense of the dystonias: the practical checklists.

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, implying that successive generations manifest the disease at an earlier age, and in more severe forms (genetic anticipation). You may read more on HD in the previous blog post titled What are the prospects of stamping out Huntington’s disease? 

Motor neurone disease (MND) 

Also known as Amyotrophic lateral sclerosis (ALS), MND is simply devastating. Recognising no anatomical boundaries, it ravages the central and peripheral nervous systems equally. MND creeps up on the neurones and causes 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 no wonder that one of the most read post on this blog is titled Is neurology research finally breaking the resolve of MND? Other previous blog posts on MND are The emerging links between depression and MNDWhat is the relationship of MND and cancer?Does diabetes protect from MND?, and MND and funeral directors-really?

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 contenders vying for the cause of MS, but the reason nerves in the central nervous system inexplicably lose their myelin sheaths remains elusive. You may read more on MS in these blog posts: The emerging progress from the world of MS , What are the remarkable drugs which have transformed the treatment of MS?, and Is low vitamin D a cause of multiple sclerosis?

Muscular dystrophy 

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 be more familiar with late onset muscular dystrophies such as Myotonic dystrophy and Facioscapulohumeral muscular dystrophy (FSHD). Read more on muscular dystrophy in these previous blog posts: How is neurology stamping out the anguish of Duchenne? and The A–Z of limb girdle muscular dystrophy (LGMD).

Rabies

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, popularised by the Steven King book and film, Cujo. Rabies manifests either as the encephalitic (furious) or the paralytic (dumb) forms. It wreaks havoc by causing irritability, hydrophobia (fear of water),  excessive sweating, altered consciousness, and inevitably death. Whilst there are vaccines to protect against rabies, a cure has eluded neuroscientists. This blog is yet to do justice to rabies but it is, at least, listed in the post titled What are the most iconic neurological disorders? But you could better by checking neurochecklists for details of the clinical features and management of rabies.

Spinal cord injury

Nothing is quite as heart-wrenching as the sudden loss of body function that results from spinal cord trauma. This often causes 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. You may read about the heroic efforts to treat spinal cord injury in the blog posts titled 6 innovations in the treatment of spinal cord injury and Head transplant, anyone?

Tetanus

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. Tetanus is caused by 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. Check neurochecklists for more on the pathology, clinical features, and management of tetanus.

 

Light brain. Mario D’Amore on Flikr. https://www.flickr.com/photos/kidpixo/3470448888

As for all lists, this will surely be subject to debate, or perhaps some healthy controversy. Please leave a comment.

6 thoughts on “The 13 most dreadful neurological disorders…and the groups standing up to them

  1. Fascinating…you don’t include Parkinson’s then? I suppose it’s just not gruesome enough!
    As a care-giver watching my beloved; a vibrant man who lost his ‘self’, his business, his motivation, his confidence, his raison d’etre and withdrew from his kids – this was just the beginning. Then reduced from first a quivering wreak to a frozen mess by this usurper in the last 18 years, to bear witness to the appalling indignities that all too slowly scrape away at everything. As his body looses more and more autonomic control and his dependency upon me blows away any shred of hope, I too am lost. We’re both exhausted and we may have as long as 15 more years of this – whoopee!
    Then he’ll die. Slowly…miles more to go! It will be the incessant choking that will cause the final insult, and he’ll be RELIEVED.
    It may not be sexy enough to make your list, but the great thing about Parkinson’s is you can guarantee decades of degradation and suffering before you die ‘with it.’
    Only one thing worse – all the 20 year-olds who are getting Parkinson’s….

    Like

    1. Dear Lisa, Many thanks for your comments which graphically emphasise the burden of Parkinson’s disease. I understand your views and appreciate how dreadful Parkinson’s disease is. It was indeed a struggle to decide what to keep out of this list. Epilepsy, Stroke, and Parkinson’s disease were particularly difficult to exclude. Unfortunately in this particular blog post I restricted myself to 13; a more complete list of dreadful neurological diseases is probably an impossible task. As I stated in the piece, this list does not intend to belittle the excluded conditions. Unfortunately any list of this nature is bound to fall short in some way. I however consoled myself that I had given prominence to these three disorders in several previous blog posts. I hope you will understand and forgive any lapses in this case

      Liked by 1 person

      1. Thanks Lisa. I expected the list to be a double-edged sword as my conclusion indicated. Thanks for understanding. I think all diseases challenge us, to bring the best out of us, and keep that milk flowing endlessly

        Liked by 1 person

  2. I feel empathy for Lisa and many others who have Parkinson’s disease, or they have a loved one that suffers with Parkinson’s disease. You can’t put one disease above the other, that I know. It really comes down to what you or your loved one is suffering from.

    So many people fear one thing…Cancer. Those people have never even thought of Neurological diseases or disorders. When they do find out about this they think, at least they have some hope with cancer.

    When I was first diagnosed with MS, my brother-in-law told my sister, “If he had to have something, MS wasn’t such a bad disease”. He of course was born into a family with plenty of money, had made good choices with investments and his career. That makes a world of difference if you become disabled. He obtained his knowledge by going online one night. He never asked me any questions or spent any time with me.

    I am Blessed because it could always be worse! I
    had just been divorced for 15 months. At least 75 % of
    the reason for the divorce was due to my health problems which no doctor was able to figure out the underlying problem. One year after my 3rd child I began with various symptoms of severe headaches with nausea and vomiting, acute and chronic sinus infections, severe fatigue, fibromyalgia symptoms…none of my doctors believed in that as a diagnosis, and numbness in my toes and feet. I started having numerous UTI’S, Cystitis and trigonitis. I had been to see Neuologist, ENT’S, Interns,
    Chiropractors, Infectious
    diseases doctor due to sinus infections (MRSA) and other bacterial and fungal infections. I finally went to a Podiatrist because I was tripping over my own two feet and missing steps! He was the only one to tell me I needed to be checked out for MS!
    When I went to my family doctor, he laughed and said I did not have MS! He refused to do any tests. I had also gone to a Pain Specialist who helped me out with chronic pain after many psychological tests and evaluations that said I had true pain. That is when I was helped with the pain.
    That gave me the strength and fortitude to want to continue living.
    My husband knew I was in pain, but couldn’t live with the different “me”. I had raised our 3 children…the youngest was 14 years old.
    That was when he wanted the divorce and forced me to proceed. I was left with no insurance, no car, etc.
    After the stress of the divorce, moving with my 2 girls, working full time plus so much overtime to keep my job (no OT pay, to get time off for time worked, which he revoked once I was diagnosed with MS!),
    I finally collapsed while working with an infant and his mother standing next to me! I had explained to the doctor I was working for that my Rt leg was dragging and my Rt arm didn’t feel nor respond as it should! His response was to do the best I could! You see what happened! I couldn’t get up from the floor! Neither leg worked! I was in a fog, but afraid for her to call out to the doctor I was working for. When I finally did, he put me in a chair and
    “We only have another hour, you answer the phone and I’ll have the receptionist bring the patients back and weigh them. Just show me how do do the shots!”. Stupidly I did it. He set me up with an internist for the next day.
    She couldn’t find anything wrong, so she sent me to every specialist which all checked out fine. The last appointment was with a Neuologist who examined me, then did brain MRI’s with and without dye. Of course the diagnosis was MS and he wanted me to go to the MS Center at the Augusta Medical University Hospital or Shepherd MS Clinic in Atlanta. All of this was over 20 years. Then I was diagnosed with Epilepsy. It is controlled by medication. My journey continues as do so many others who have Neurological Disorders/Diseases.

    Liked by 1 person

    1. Thank you for sharing your experience Barbara. I agree that the personal journey plays a big role in how distressing any disease turns out to be. My respect goes out to you and everyone coping with the burden of neurological diseases. Your strength can only be imagined but not fully appreciated. Thanks for your insightful comments.

      Liked by 1 person

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