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Vestibular Disorders and Rehabilitation
Published in R James A England, Eamon Shamil, Rajeev Mathew, Manohar Bance, Pavol Surda, Jemy Jose, Omar Hilmi, Adam J Donne, Scott-Brown's Essential Otorhinolaryngology, 2022
The concepts of phobic positional vertigo, space motion discomfort, and chronic subjective dizziness were brought together to produce the entity known as persistent perceptual postural dizziness (PPPD), referring to dizziness, unsteadiness, or non-spinning vertigo that are present pervasively over 3 months or more and that are exacerbated by upright posture, active or passive movement, and exposure to moving or complex visual stimuli and thought to be a functional disorder. Models for explaining PPPD have been drawn from anatomical, neurochemical, and cognitive behavioural theories, and it is considered to be a functional neurological disorder.3 Anxiety and major depressive disorders are common in patients with PPPD but do not occur in all cases.
Clinical presentation and differential diagnosis of dementia in younger people
Published in Marjolein de Vugt, Janet Carter, Understanding Young Onset Dementia, 2021
Yolande A.L. Pijnenburg, Cynthia Klaassen
Logopenic Progressive Aphasia (LPA) was first described by Gorno-Tempini et al. as a distinct clinical syndrome within the spectrum of Primary Progressive Aphasia (PPA) syndromes (Gorno-Tempini et al., 2004; Mesulam, 2001). It is characterised by word-finding difficulties and pauses in spontaneous speech, together with impaired repetition of sentences and words. Whereas the other two prototypical syndromes, the semantic variant and the nonfluent variant of PPA, are characterised by underlying frontotemporal dementia, the underlying pathology of LPA is Alzheimer's disease in the majority of cases (Gorno-Tempini et al., 2011). According to the definition of PPA, a language problem is the first and most prominent deficit over a longer period of time, and the principal cause of daily living impairment (if present at all), before disturbances in other cognitive domains and/or behaviour may occur. Whereas the semantic and nonfluent variants of PPA display more or less homogenous clinical pictures, the concept of LPA is more heterogeneous. Moreover, working memory seems to be implicated in the impaired repetition of longer sentences. More recently, ‘mixed’ or LPA Plus syndromes have been described by several authors, delineating that other language functions, such as comprehension and semantic knowledge, may accompany LPA features in Alzheimer's disease (Leyton et al., 2015; Louwersheimer et al., 2016). The main differential diagnoses of LPA are a functional neurological disorder and a cerebrovascular accident. Although the latter should be identified on neuroimaging, LPA is sometimes interpreted as a deficit after a TIA or stroke. It is the gradual progressive course that eventually leads to the correct diagnosis.
Neuropsychiatry in Film
Published in Eelco F. M. Wijdicks, Neurocinema—The Sequel, 2022
Functional neurology is diagnosed in patients who have neurologic-type symptoms (inability to speak, move, or feel or a persistent muscle contraction) but without brain tissue or physiological change. The incidence is comparable to many known neurologic illnesses and is a very common observation in neurology clinics and epilepsy monitoring units. Preceding stressors are no longer a requirement in the diagnostic criteria for functional neurologic disorder, but with hindsight, it can be linked to a triggering event in many patients. Before “functional neurology” became the accepted term, Charcot started the diagnostic inquiry into functional symptoms and diagnosed “hysteria” if there were convulsive crises with the characteristic poses such as arc-en-cercle or if patients presented with persistent neurologic deficits without an identifiable substrate. Charcot divided hysteria into four phases—epileptoid, clownism (i.e., contortions and acrobatic postures), postures indicating passion and desire (“délire érotique”), and delirium-hallucinations, which he called “Grande Hystérie.” Numerous labels have been applied over the years for more specific manifestations such as seizures, ranging from “hystero-epilepsy” in the 19th century to “non-epileptic attack disorder” in the late 20th century. For Charcot, hysteria was primarily a hereditary disorder. (Freud later added sexual abuse fantasies in females.) Charcot knew too well that hysteria was distinct from malingering. He also recognized physical trauma could cause “hysteria” and more in males, often in muscular, virile men of the working class with only a minor visible injury (e.g., a burn on the hand causing a permanent contracture). Charcot is featured in Augustine and discussed in Chapter 2. Augustine has a functional hemiparesis that requires treatment (Figure 8.1). Charcot could not possibly film his patients, and we only have descriptions and drawings of the attacks (Charcot and Richer’s Les Démoniaques Dans l’Art). Some of his patients were photographed in a studio (Service Photographique de la Salpêtrière). A spell followed a breath-holding pallor followed by redness, neck engorgement, and upward eye deviation. Foam appeared on the lips, usually early and not during the resolution phase as in a true seizure. Initially, the muscles were completely limp, and then “acrobatics” began. Sometimes, the patient curved forward with the head and feet touching the bed, but the patient could also lie on the side. Charcot also described bizarre movements (as if wrestling with an imaginary being) and savage cries. Hissing respiration would be interrupted by hiccups, and the mouth would droop open with tongue protrusion. Charcot often described these contortions as “being possessed.” Later, in 1899, the Romanian Gheorghe Marinescu filmed a single case of “hysterical hemiplegia” cured with hypnosis (see Chapter 2). In the next century, hypnosis received more serious attention, and movie cameras allowed us to view functional disease in soldiers returning from World War I.
Atypical Guillain-Barré syndrome presenting with fluctuating weakness
Published in Baylor University Medical Center Proceedings, 2023
Lauren L. Crowther, Amarjyot K. Randhawa, Robert W. Plambeck
Thus far, the laboratory and imaging evaluations were unremarkable. Head and neck computed tomography (CT) angiography, noncontrast head CT, and C-spine, T-spine, and brain magnetic resonance imaging ruled out intracranial pathology, spinal cord lesions, stroke, and transverse myelitis. Mild elevations of the erythrocyte sedimentation rate (21 mm/h) and C-reactive protein (9.84 mg/L) were atypical of myopathic illness. Lumbar puncture demonstrated normal protein, no cells, and a negative meningitis/encephalitis panel; however, opening pressure was elevated (30 cm H2O). He was initiated on acetazolamide for intracranial hypertension, likely unrelated to his tetraplegia. Infectious disease ran an extensive unyielding infectious workup of the serum, urine, and cerebrospinal fluid (Table 1). Incidentally, treponemal antibodies were positive and penicillin was initiated, though his presentation was atypical for syphilis. After excluding organic pathology, a functional neurological disorder appeared to be the likely diagnosis.
The vicious cycle of functional neurological disorders: a synthesis of healthcare professionals’ views on working with patients with functional neurological disorder
Published in Disability and Rehabilitation, 2022
Caroline Barnett, Rebecca Davis, Claire Mitchell, Sarah Tyson
Patients with functional neurological disorder (FND) present with motor and/or sensory or cognitive neurological symptoms which are incongruous with known neurological conditions, cannot be attributed to another medical or neurological cause, and have a significant impact on patients’ daily functioning [1]. Patients with FND can present with a wide range of functional symptoms, such as limb weakness [2], tremor [3], seizures [4], communication difficulties [5], or memory difficulties [6]. Because these symptoms are neurological, patients are often referred to neurologists in order to differentially diagnose the cause of the symptoms. Ideally, they would then be managed by a multidisciplinary team of neurologists, psychologists, psychiatrists, nurses and allied health professionals. However, patients with FND often receive inequitable care and may not receive multidisciplinary intervention [7].
Prognostic factors in non-organic hearing loss in children
Published in International Journal of Audiology, 2022
Christina Pflug, Saskia Kiehn, Jana-C. Koseki, Hans Pinnschmidt, Frank Müller, Julie C. Nienstedt, Till Flügel, Almut Niessen
Our results do not only suggest that the prescription of hearing aids seems to be unintentionally led by age and gender of the child. They also highlight the negative impact of hearing aids on the outcome, as they lower the chance of full recovery from the NOHL significantly. This is the first reason why we strongly advise against providing hearing aids in NOHL. The second reason is possible noise damage that may be caused when children with no sensorineural hearing loss use hearing aids. While there is much debate over the use of hearing aids in mild hearing loss (Johnson 2018; Lewis 2020), no case can be made that hearing aids are to be administered in children in cases of normal organic hearing. Finally, the third reason why we advise against hearing aids in NOHL is that prescribing hearing aids may confirm and support the child’s belief of not hearing well by suggesting there was an organic illness. A somatic symptom is inadvertently preserved; underlying psychological or social stressors are not adequately addressed, as would be appropriate in a functional neurological disorder.