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Advanced Neurology                                                   POTS with tics versus tic-like behaviors



            POTS and plays a key role in the treatment approach   The exact pathophysiology of neurodevelopmental tics
            to POTS, with the identification of a wide range of   remains elusive, despite recent advances in understanding
            predisposing, precipitating and perpetuating factors.   the sensorimotor processes behind the urge to tic and
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            Pharmacotherapy also plays a role in the treatment of   tic expression.  Dopaminergic neurotransmission is
            POTS: corticosteroids (fludrocortisone), alpha-agonists   primarily implicated, although other neurotransmitter
            (midodrine), beta-adrenergic blockade, ivabradine, and   systems are believed to be involved. For example, research
            – less commonly – immunotherapy, have been used    using animal models, such as knockout mice, has suggested
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            for neuropathic POTS.  However, the overall effects of   a potential link to histidine decarboxylase deficiency.
                               3
            pharmacological therapy are modest. 2              Both genetic and environmental factors are thought to
                                                               contribute to the onset of GTS, with studies indicating a
            1.2. Neurodevelopmental tics and Gilles de la      heritability rate of 0.77. 26
            Tourette syndrome (GTS)
                                                               1.3. Functional tic-like behaviors
            Neurodevelopmental tics are sudden, rapid, recurrent,
            and non-rhythmic movements (motor tics) or         Functional tic-like behaviors are a subgroup of functional
            sounds (vocal tics), resulting from altered pathways   neurological  disorder with  motor  manifestations
            of brain development.  Tic disorders are the most   that resemble neurodevelopmental tics and typically
                                13
            common hyperkinetic disorder in children.  GTS     involve a complex clinical phenomenology, ranging
                                                    14
            is characterized by multiple tics as well as comorbid   from  repetitive twitching and jerking movements  to
            psychiatric disorders – especially obsessive-compulsive   sounds and meaningful  vocalizations. 27,28  By definition,
            disorder and attention-deficit  hyperactivity disorder   these clinical manifestations are not consistent with
            (ADHD)  – in  about  90% of  cases. 13,15-17   The multiple   a neurodevelopmental pathway and often correlate
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            phenotypes of GTS can affect different aspects of   with psychological stressors.  Functional tics have
            patients’ health-related quality of life across the   traditionally been considered a condition with a relatively
            lifespan. 18,19  The prevalence of GTS in youth ranges   low prevalence, compared to other functional motor
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            from 0.3% to 1%, while isolated tics affect up to 5% of   symptoms.  Comparisons with data collected before
            the general population at any point in life.  In most   the COVID-19 pandemic show that the prevalence
                                                 14
                                                               of  functional tics in  adolescents  and  young adults has
            cases, the onset of neurodevelopmental tics occurs in   increased dramatically since the pandemic. 29,31
            childhood, between the age of 5 and 7, with a male-to-
            female ratio of about 3:1 – 4:1. 20                  Despite their resemblance to neurodevelopmental tics,
                                                               the  clinical  presentation  of functional tic-like behaviors
              Neurodevelopmental tics tend to spread to different   reported during the COVID-19 pandemic has some peculiar
            body parts following a rostrocaudal direction over the   characteristics, which can assist the differential diagnosis.
            years following their onset.  The most common simple   Functional tics are mainly diagnosed in adolescents and
                                   20
            motor  tics  include  eye  blinking,  facial  grimacing,  and   young adults, with a significant preponderance of the
            shoulder shrugging, while throat clearing, sniffing, and   female gender. In most cases, there is no family history of
            grunting are examples of simple vocal tics. More complex   tics. 28,30  Both case series 32-34  and controlled studies 35-37  have
            motor tics involve multiple body parts and even resemble   yielded consistent findings about phenotypical differences
            intentional actions, such as palilalia (repeated gestures)   between the two conditions. Functional tic-like behaviors
            and echopraxia (mimicking others’ movements).      usually present with an acute or subacute onset, while
            Complex  vocal  tics involve  full words  or phrases,  such   neurodevelopmental tics have a gradual onset following
            as palilalia (repetition of own words) and echolalia   a rostrocaudal distribution.  Moreover, the clinical
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            (repetition of others’ words). Socially inappropriate   phenomenology of functional tic-like behaviors is more
            language  (coprolalia)  or  gestures  (copropraxia)  are   varied, with repetitive movements involving the limbs
            reported by a minority of patients (10 – 30%), typically   and a higher prevalence of self-injurious tics, such as head
            in association with a range of more simple motor and   banging or self-hitting.  Complex vocal tics in the form of
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            vocal tics.  A hallmark of neurodevelopmental tics is   random words or swear words (coprolalia) are frequently
                    21
            the presence of premonitory urges, which are distressing   part of the repertoire of functional tic-like behaviors, while
            sensory experiences that precede the expression of   they are reported by a subset of patients with GTS or other
            tics.  The sensory component is relevant to both   neurodevelopmental tic disorders. 21,28,32,38  Data from a
               22
            diagnosis and treatment since awareness of these urges   recent cross-sectional study showed that both simple head
            is often a prerequisite of behavioral interventions for   movements such as neck jerking and complex vocalizations
            neurodevelopmental tics. 23                        (words/full sentences) were more likely to be reported by


            Volume 4 Issue 2 (2025)                        102                               doi: 10.36922/an.8525
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