Cognitive, Graphomotor, and Psychosocial Challenges in Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections (PANDAS).

OBJECTIVES: Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS) is characterized by the sudden onset of obsessive-compulsive disorder (OCD) and other neurobehavioral symptoms following group A streptococcal infection. The cardinal neuropsychiatric symptoms are believed to reflect an aberrant autoimmune or inflammatory response that may selectively disrupt basal ganglia function. The investigators examined whether neuropsychological skills associated with frontostriatal networks (executive functions and motor skills) are affected in patients with PANDAS following resolution of acute symptoms and the degree to which there are persistent social, emotional, and academic difficulties. METHODS: Twenty-seven patients ages 6-14 years (mean age=9.63 years [SD=1.78]; male, N=22) completed neuropsychological testing as part of routine clinical care. Performances on measures of intellectual ability, executive function, motor skills, and academic skills are reported, as well as parent-reported emotional, behavioral, and social skills. RESULTS: On neuropsychological measures, patients exhibited average intellectual functioning with relative and mild difficulties in skills supporting cognitive efficiency, including attentional regulation, inhibitory control, and processing speed. Dexterity was normal but graphomotor skills were reduced. Core reading, math, and writing skills were within expectations, but reading and math fluency were reduced, and the majority of patients received special education services or accommodations. Parents reported high levels of concern about anxiety, depression, inattention, hyperactivity, and social skills. CONCLUSIONS: These findings indicated relative difficulties with aspects of executive and motor functions. Although evaluations were performed following the resolution of acute symptoms, ongoing and significant academic difficulties and emotional, behavioral, and social concerns were targets for clinical intervention and support.

Isolated Cervical Dystonia: Management and Barriers to Care.

Background: Cervical dystonia (CD) is a rare disorder, and health care providers might be unfamiliar with its presentation, thus leading to delay in the initial diagnosis. The lack of awareness displays the need to highlight the clinical features and treatment in cervical dystonia. In our cohort, we have identified an earlier age of onset in men, despite an overall preponderance of affected women. Objective: We aim to identify the prevalence, age of onset, spread, and treatment modalities of CD in the population. We also highlight the barriers which patients encounter related to diagnosis, follow-up, and treatment. Methods: We reviewed 149 CD patients who attended specialized Dystonia Clinics over a 14-year period. Dystonia severity was rated using the Burke-Fahn-Marsden (BFM), Tsui, and Toronto Western Spasmodic Torticollis Rating Scales (TWSTRS). Mood and quality of life were assessed using Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), and 36-Item Short Form Health Survey (SF-36). Results: CD patients were majority White (91.3%) and more commonly female (75.8%). Men had an earlier median age of onset, 40.5 years (p = 0.044). BAI revealed a mean score of 7.2 (±6.4, n = 50) indicating minimal anxiety while BDI revealed a mean score of 7.30 (±7.6, n = 50) indicating minimal depression. The only SF-36 subscales associated with CD severity were physical functioning (p = 0.040) pain (p = 0.008) and general health (p = 0.014). Conclusion: There appear to be gender differences in both the prevalence and age of onset of the disease. There was a 3-fold higher incidence in women than in men. CD patients of both sexes experience barriers to care, which can be reflected in their quality of life and time-to-diagnosis. In addition, males were less likely to experience an objective benefit with botulinum toxin treatment and more likely to discontinue care. Greater awareness of CD by health care providers is important to reduce the time-to-diagnosis.

Cognitive Complaints in Motor Functional Neurological (Conversion) Disorders: A Focused Review and Clinical Perspective.

Functional neurological (conversion) disorder (FND) is a neuropsychiatric condition characterized by sensorimotor symptoms exhibiting features incompatible with other neurologic diseases. Individuals with motor FND (mFND) typically present with limb weakness, nonepileptic seizures, and/or abnormal movements. However, this population also frequently reports clouded thinking, inattention, and memory difficulties. Cognitive complaints in individuals with mFND are important to evaluate as they may negatively impact quality of life and impede treatment engagement. We provide a narrative review of the neuropsychological testing literature detailing neurocognitive profiles of individuals with mFND. We also present three illustrative clinical cases at the intersection of mFND and cognitive concerns. Several studies and our case examples highlight that generally normal cognitive performance can be observed concurrently with subjective cognitive complaints in some individuals with mFND; this mismatch may be a possible "rule-in" sign of functional cognitive symptoms. Other studies have reported impairments in attention, memory, language, visuospatial, and executive functioning in individuals with mFND. These impairments could be related to medical-psychiatric comorbidities, psychotropic medication side effects, and intrinsic disease mechanisms. When evaluating individuals with mFND and their cognitive complaints, clinicians can use performance validity test and psychopathology findings to help them interpret the neuropsychological test results. Perceptual mismatches between intact objective cognitive performance and subjective cognitive complaints may reflect a negative attentional bias for cognitive abilities that can be targeted with cognitive retraining and cognitive behavioral therapy. Neuropsychological evaluations may provide a useful adjunctive tool clinicians can use to help assess individuals with mFND and cognitive concerns.

Sensory Processing Difficulties in Functional Neurological Disorder: A Possible Predisposing Vulnerability?

BACKGROUND: Functional neurological disorder (FND) is a prevalent neuropsychiatric condition characterized by sensorimotor difficulties. Patients with FND at times report that sensory experiences trigger and/or exacerbate their symptoms. Sensory processing difficulties are also commonly reported in other psychiatric disorders frequently comorbid in FND, suggesting that contextualizing sensory profiles in FND within a biopsychosocial model may be clinically relevant. OBJECTIVE: To address this literature gap, we conducted a retrospective cohort study to examine sensory processing patterns and their relationship to other neuropsychiatric characteristics in patients with FND. METHODS: A retrospective chart review design was used to investigate sensory processing patterns, established with the Adolescent/Adult Sensory Profile self-report questionnaire, in 44 patients with FND. Univariate analyses of cross-sectional screening tests followed by multivariate linear regression analyses were performed to identify clinical factors associated with sensory processing scores in the FND cohort. RESULTS: Compared to normative data, most patients with FND reported sensory processing tendencies toward low registration, sensory sensitivity, and sensation avoiding. In multivariate regression analyses, the presence of a lifetime anxiety disorder independently predicted elevated low registration scores, while female gender and number of current medications independently predicted increased sensory sensitivity scores. In uncorrected univariate analyses only, individuals with psychogenic nonepileptic seizures were more likely to report increased sensory sensitivity and elevated low registration. CONCLUSION: These preliminary findings support sensory processing difficulties in some patients with FND. Prospective and large sample size studies are needed to investigate relationships between sensory processing profiles and neuropsychiatric comorbidities, FND subtypes, and treatment outcomes.

Briquet syndrome revisited: implications for functional neurological disorder.

With the creation of the Somatic Symptom and Related Disorders category of the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition in 2013, the functional neurological (symptom) disorder diagnostic criteria underwent transformative changes. These included an emphasis on 'rule-in' physical examination signs/semiological features guiding diagnosis and the removal of a required proximal psychological stressor to be linked to symptoms. In addition, the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition somatization disorder, somatoform pain disorder and undifferentiated somatoform disorder conditions were eliminated and collapsed into the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition somatic symptom disorder diagnosis. With somatic symptom disorder, emphasis was placed on a cognitive-behavioural (psychological) formulation as the basis for diagnosis in individuals reporting distressing bodily symptoms such as pain and/or fatigue; the need for bodily symptoms to be 'medically unexplained' was removed, and the overall utility of this diagnostic criteria remains debated. A consequence of the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition restructuring is that the diagnosis of somatization disorder that encompassed individuals with functional neurological (sensorimotor) symptoms and prominent other bodily symptoms, including pain, was eliminated. This change negatively impacts clinical and research efforts because many patients with functional neurological disorder experience pain, supporting that the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition would benefit from an integrated diagnosis at this intersection. We seek to revisit this with modifications, particularly since pain (and a Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition somatization disorder comorbidity, more specifically) is associated with poor clinical prognosis in functional neurological disorder. As a first step, we systematically reviewed the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition somatization disorder literature to detail epidemiologic, healthcare utilization, demographic, diagnostic, medical and psychiatric comorbidity, psychosocial, neurobiological and treatment data. Thereafter, we propose a preliminary revision to the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition allowing for the specifier functional neurological disorder 'with prominent pain'. To meet this criterion, core functional neurological symptoms (e.g. limb weakness, gait difficulties, seizures, non-dermatomal sensory loss and/or blindness) would have 'rule-in' signs and pain (>6 months) impairing social and/or occupational functioning would also be present. Two optional secondary specifiers assist in characterizing individuals with cognitive-behavioural (psychological) features recognized to amplify or perpetuate pain and documenting if there is a pain-related comorbidity. The specifier of 'with prominent pain' is etiologically neutral, while secondary specifiers provide additional clarification. We advocate for a similar approach to contextualize fatigue and mixed somatic symptoms in functional neurological disorder. While this preliminary proposal requires prospective data and additional discussion, these revisions offer the potential benefit to readily identify important functional neurological disorder subgroups-resulting in diagnostic, treatment and pathophysiology implications.