Animals in the Brain

Mulroy, Eoin; Balint, Bettina; Adams, Matthew; Campion, Tom; Merello, Marcelo; Bhatia, Kailash P.

doi:10.1002/mdc3.12734

Cited by 9 publications

(5 citation statements)

References 24 publications

(40 reference statements)

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“…15 MRI abnormalities including basal ganglia, thalamus and brainstem T2-weighted hyperintensities and in more advanced cases, the typical "face of the giant panda" or "face of the panda cub" signs may be apparent. 14,16 The approach to diagnosis of WD depends on the clinical presentation, as the sensitivity and specificity of diagnostic tests vary in different scenarios. Numerous guidelines have been published to aid clinicians in this regard.…”

Section: Wilson's Diseasementioning

confidence: 99%

“…Psychiatric disturbances(personality and behavioral changes, psychosis) and cognitive impairment often associate with MD 15 . MRI abnormalities including basal ganglia, thalamus and brainstem T2‐weighted hyperintensities and in more advanced cases, the typical “face of the giant panda” or “face of the panda cub” signs may be apparent 14,16 …”

Section: Combined Involvement Of the Nervous System And Liver From A Single Disease Entitymentioning

confidence: 99%

“… 15 MRI abnormalities including basal ganglia, thalamus and brainstem T2‐weighted hyperintensities and in more advanced cases, the typical “face of the giant panda” or “face of the panda cub” signs may be apparent. 14 , 16 …”

Section: Combined Involvement Of the Nervous System And Liver From A Single Disease Entitymentioning

confidence: 99%

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Movement Disorders and Liver Disease

Mulroy

Baschieri

Magrinelli

et al. 2021

Movement Disord Clin Pract

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The association of movement disorders with structural or functional hepatic disease occurs in three principal scenarios: (1) combined involvement of both organ systems from a single disease entity, (2) nervous system dysfunction resulting from exposure to toxic compounds in the setting of defective hepatic clearance, or (3) hepatic and/or neurological injury secondary to exposure to exogenous drugs or toxins. An important early step in the workup of any patient with combined movement disorders and liver disease is the exclusion of Wilson's disease. Diagnostic delay remains common for this treatable disorder, and this has major implications for patient outcomes. Thereafter, a structured approach integrating variables such as age of onset, tempo of progression, nature and severity of liver involvement, movement disorder phenomenology, exposure to drugs/toxins and laboratory/neuroimaging findings is key to ensuring timely diagnosis and disease-specific therapy. Herein, we provide an overview of disorders which may manifest with a combination of movement disorders and liver disease, structured under the three headings as detailed above. In each section, the most common disorders are discussed, along with important clinical pearls, suggested diagnostic workup, differential diagnoses and where appropriate, treatment considerations.The association of movement disorders (MD) with hepatic disease is a noteworthy one. In some instances, it is merely coincidental. In others, it may reflect:

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Section: Wilson's Diseasementioning

confidence: 99%

Section: Combined Involvement Of the Nervous System And Liver From A Single Disease Entitymentioning

confidence: 99%

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Movement Disorders and Liver Disease

Mulroy

Baschieri

Magrinelli

et al. 2021

Movement Disord Clin Pract

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“…Some IEMs have striking MRI abnormalities, for example abnormalities of the white matter in patients with leukodystrophies [35]. In patients with PKAN an "eye-of-the-tiger" sign can be seen, which is caused by the accumulation of iron in the globus pallidus, and in patients with Wilson's disease a "face-of-the-giant-panda" sign can be found [36]. However, many MRI abnormalities in IEMs are not very specific, including atrophy and (vascular) white matter lesions, and may overlap with other more common disorders.…”

Section: Bloodmentioning

confidence: 99%

How to detect late-onset inborn errors of metabolism in patients with movement disorders – A modern diagnostic approach

Koens

Vries

Vansenne

et al. 2021

Parkinsonism & Related Disorders

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We propose a modern approach to assist clinicians to recognize and diagnose inborn errors of metabolism (IEMs) in adolescents and adults that present with a movement disorder. IEMs presenting in adults are still largely unexplored. These disorders receive little attention in neurological training and daily practice, and are considered complicated by many neurologists. Adult-onset presentations of IEMs differ from childhood-onset phenotypes, which may lead to considerable diagnostic delay. The identification of adult-onset phenotypes at the earliest stage of the disease is important, since early treatment may prevent or lessen further brain damage. Our approach is based on a systematic review of all papers that concerned movement disorders due to an IEM in patients of 16 years or older. Detailed clinical phenotyping is the diagnostic cornerstone of the approach. An underlying IEM should be suspected in particular in patients with more than one movement disorder, or in patients with additional neurological, psychiatric, or systemic manifestations. As IEMs are all genetic disorders, we recommend next-generation sequencing (NGS) as the first diagnostic approach to confirm an IEM. Biochemical tests remain the first choice in acute-onset or treatable IEMs that require rapid diagnosis, or to confirm the metabolic diagnosis after NGS results. With the use of careful and systematic clinical phenotyping combined with novel diagnostic approaches such as NGS, the diagnostic yield of late-onset IEMs will increase, in particular in patients with mild or unusual phenotypes.

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“…El más famoso es el referido al mesencéfalo en las imágenes axiales de resonancia magnética (RM) craneal en los pacientes con el parkinsonismo atípico más frecuente: la parálisis supranuclear progresiva. Las orejas de Mickey son los pedúnculos cerebrales y la cara es el mesencéfalo atrófico 4 (Fig. 1).…”

Section: Neuroimagen Y Dibujos Animadosunclassified

Los dibujos animados como vehículo docente en neurología

Gómez¹

2021

KRANION

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Los dibujos animados, además de servir para el entretenimiento de los niños, pueden ser usados para la docencia de la neurología. Tenemos ejemplos en neuroimagen como el «signo de Mickey Mouse» en la resonancia magnética de la parálisis supranuclear progresiva. Si nos centramos en la semiología es más fácil explicar la amnesia anterógrada ayudándonos de Dory, de Buscando a Nemo y las diferentes alteraciones del lenguaje, como los síndromes del idioma extranjero y acento extranjero vía Buzz Lightyear, y de la articulación gracias a Porky (disfemia) y al Pato Donald (disartria). Además, Silvestre y Piolín han jugado un papel importante en el estudio del lenguaje gestual. Sobre los gestos, la mano de la Familia Addams es útil para entender el fenómeno de la mano alienígena o ajena. Si queremos explicar enfermedades, Popeye nos ayuda a entender la clínica de la distrofia facio-escápulo-humeral, Pluto y la bella durmiente muestran fielmente trastornos del sueño, el Jorobado de Notre-Dame ejemplifica la neurofibromatosis tipo I y con Clara, la amiga de Heidi, podemos explicar la paraplejia funcional. En relación con enfermedades causadas por dibujos animados cabe considerar un capítulo de Pokémon que causó múltiples crisis epilépticas en Japón, de utilidad para explicar la epilepsia fotosensible. Los dibujos animados pueden ayudarnos a explicar las enfermedades neurológicas y su semiología.

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Animals in the Brain

Cited by 9 publications

References 24 publications

Movement Disorders and Liver Disease

Movement Disorders and Liver Disease

How to detect late-onset inborn errors of metabolism in patients with movement disorders – A modern diagnostic approach

Los dibujos animados como vehículo docente en neurología

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