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This chapter gives a brief history of Huntington’s disease (HD) starting with the original description by George Huntington. The prevalence of HD is discussed together with data variations in different parts of the world. For example the incidence of HD in Europe and North America is approximately 1 person in 10,000. Apart from humans, no other animal naturally develops HD. The condition can start at almost any age but it is more frequent in midlife. The duration of the disease can be very variable but 20 years is often given as an approximate average.

This chapter focuses mainly on the movement disorder, speech, balance, weight loss, swallowing, and speech. Chorea or purposeless involuntary movement is most commonly associated with HD. A patient may have a mixture of movement problems such as dystonia (abnormal posture), and bradykinesia (slowness of movement). The pattern of movement disorder may vary between people but frequently the dystonia and bradykinesia become more prominent as the disease progresses. As this happens more professionals may become involved in the care. It is important for the carer(s) to also take care of themselves.

Juvenile Huntington’s disease (JHD) is rare. It is usually defined as someone having an onset before 21 years. A more accurate description would be juvenile-onset HD. The origins of this definition are obscure but the distinction has been recognized for many years. The proportions of JHD cases may vary in different parts of the world but a figure of approximately five per cent is useful. JHD is not a separate category but rather part of the spectrum. Whilst it is still useful to think about the needs of young people in terms of the different ways they can present to a doctor some of the limitations of the definition will be explored. As part of the process of developing new drugs the European Medicines Agency (EMA) would like information on how the drug affects children. The term JHD does not cover this accurately so new term, paediatric Huntington’s disease (PHD) has been devised so that young people under the age of 18 years and currently affected by HD can be identified.

Renegade physician Paracelsus compared St. John’s Day dances to earthquakes, epileptic tremors, and tics. This ecosophical and vitalist concept, according to which all sorts of bodies echo one another’s shaking motions, countered long-held academic prejudice against witchcraft; neither choreomaniacs nor witches were subject to supernatural forces. Rather, the ‘vital spirits’ caused limbs, like branches, to shake. What’s more, dancing was now thought to cure dancing, and municipal authorities keen to keep a Strasbourg dancing mania in check employed guards to help wear dancers out—while exorcism associated religious, municipal, and medical experts. The translatio or passage from collective to individual disorder, epitomized in St. Vitus, now patron saint of all dance maniacs, continued throughout the eighteenth and nineteenth centuries, as neurologists’ theories of chorea, epilepsy and insanity aligned popular carousing with individual quaking motions. Choreomania came to signal the epidemic proliferation of what Giorgio Agamben has styled purposeless gesture.

Modernity can be understood as the cultivation of a fantasy about the past: in the case of choreomania, this ‘past’ was imagined as a rumbling horde, a Bacchic chorus against which purposeful ‘modernity’ positioned itself. Meaningless gesture, after Agamben, epitomized in the nineteenth century in the figure of ‘chorea’, similarly became with the scientific discourse on choreomania a manner of thinking modernity’s double movement, forward and back, between efficient industrialization and the collective archaicity out of which this efficiency imagined itself to arise. Reperforming my own archival return to the still ‘living’ site of the Echternach dancing procession, I discover that the ever more institutionalized event highlights the power of choreopolitical organization displayed by the nation state. As in Meige’s day, the procession has been evacuated of nervous disorder or bodily disruption. My archival reperformance reveals an experience of historicity shorn of a historical encounter.

It is much better, people think, for the nerves than the mind to be ill. The nerves are physical structures, and heal in the way that all organs of the body heal naturally. Disorders of the mind are frightening because they are so intangible, and, we think, may well lead to insanity rather than recovery. From time out of mind, people have privileged nervous illness over mental illness. From time out of mind, societies have had expressions for the varieties of frets, anxieties, and dyspepsias to which the flesh is heir. In France and England in the seventeenth and eighteenth centuries, one term was “vapours,” a reference from humoral medicine to supposed exhalations of the viscera that would rise in the body to affect the brain. A major apostle was London physician John Purcell, writing in 1702, of “those who have laboured long under this distemper, [who] are oppressed with a dreadful anguish of mind and a deep melancholy, always reflecting on what can perplex, terrify, and disorder them most, so that at last they think their recovery impossible, and are very angry with those who pretend there is any hopes of it.” He emphasized melancholia and anguish, and for him the “vapours” were something more than a mild attack of the frets. But this was not for everyone. Lady Mary Wortley Montagu, now 60 and living in exile in Italy, described to her estranged husband in 1749 Italian health care arrangements, and how physicians visited rich and poor alike. “This last article would be very hard if we had as many vapourish ladies as in England, but those imaginary ills are entirely unknown here. When I recollect the vast fortunes raised by doctors amongst us [in England], and the eager pursuit after every new piece of quackery that is introduced, I cannot help thinking there is a fund of credulity in mankind . . . and the money formerly given to monks for the health of the soul is now thrown to doctors for the health of the body, and generally with as little real prospect of success.”

■ Receives inputs from the frontal eye field (FEF), supplementary eye field (SEF), dorsolateral prefrontal cortex (DLPC), internal medullary lamina of the thalamus, and substantia nigra pars compacta (SNpc, the dopaminergic portion) ■ Projects to substantia nigra pars reticulata (SNpr) and the globus pallidus ■ Receives inhibitory inputs from the caudate nucleus ■ Sends inhibitory signals to intermediate layers of the superior colliculus Parkinson’s disease is a progressive neurodegenerative disorder that affects about 1% of adults over 60 years of age. 1. Loss of pigmented dopaminergic neurons in the SNpc 2. Presence of Lewy bodies (not specific for Parkinson’s disease) 3. Decreased dopamine reaching the striatum causes increased inhibitory output from the globus pallidus internal segment and SNpr, thereby inhibiting movement 1. Standard therapy: levadopa and carbidopa (a peripheral decarboxylase inhibitor to reduce motor fluctuations and dyskinesia) e.g., such as Sinemet or Sinemet CR 2. Monoamine oxidase-β inhibitor (e.g., Selegiline); may have neuroprotective effect 3. Dopamine agonist (e.g., bromocriptine, a D2 agonist, and apomorphine, a D1 and D2 agonist) 4. Anticholinergics (e.g., Benzhexol) 5. Amantadine (seldom used) 6. Surgery ■ Thalamotomy or thalamic deep brain stimulation of the ventral lateral nucleus to reduce medically refractory tremor. The mechanism of action is unknown; these procedures may destroy autonomous neural activity (synchronous bursts) that has the same frequency as the limb tremor. ■ Pallidotomy or pallidal stimulation to reduce contralateral dyskinesia (e.g., bradykinesia, rigidity, and tremor).