The Neuropsychiatry and Neuropsychology Of Lipoid Proteinosis

Thornton, Helena B.; Nel, Daan; Thornton, Dorothy; Honk, Jack van; Baker, Gus A.; Stein, Dan J.

doi:10.1176/jnp.2008.20.1.86

Cited by 55 publications

(41 citation statements)

References 185 publications

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“…UWD is a rare genetic developmental disorder wherein selective bilateral amygdala brain calcifications occur (33). We selected four female subjects without any history of secondary psychopathology or epilepsy from a previously described group of UWD subjects in South Africa (44) where this genetic disease is most prevalent (see also ref. 45).…”

Section: Methodsmentioning

confidence: 99%

Generous economic investments after basolateral amygdala damage

Honk

Eisenegger

Terburg

et al. 2013

Proc. Natl. Acad. Sci. U.S.A.

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Contemporary economic models hold that instrumental and impulsive behaviors underlie human social decision making. The amygdala is assumed to be involved in social-economic behavior, but its role in human behavior is poorly understood. Rodent research suggests that the basolateral amygdala (BLA) subserves instrumental behaviors and regulates the central-medial amygdala, which subserves impulsive behaviors. The human amygdala, however, typically is investigated as a single unit. If these rodent data could be translated to humans, selective dysfunction of the human BLA might constrain instrumental social-economic decisions and result in more impulsive social-economic choice behavior. Here we show that humans with selective BLA damage and a functional central-medial amygdala invest nearly 100% more money in unfamiliar others in a trust game than do healthy controls. We furthermore show that this generosity is not caused by risk-taking deviations in nonsocial contexts. Moreover, these BLA-damaged subjects do not expect higher returns or perceive people as more trustworthy, implying that their generous investments are not instrumental in nature. These findings suggest that the human BLA is essential for instrumental behaviors in socialeconomic interactions.economic trust | altruism

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Section: Methodsmentioning

confidence: 99%

Generous economic investments after basolateral amygdala damage

Honk

Eisenegger

Terburg

et al. 2013

Proc. Natl. Acad. Sci. U.S.A.

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“…7 Subsequent medial temporal lobe architectural distortion with gliotic tissue and calcium accumulation can lead to a constellation of reported neurologic manifestations, which range from migraine, variable degrees of mental retardation, seizures, depression, anxiety, and panic attacks to disturbances in decision making, memory, and abnormal social interaction patterns. 8,9 These varied symptoms frequently lead to radiologic evaluation by CT or MR imaging, which, in unsuspected patients, may indicate the proper diagnosis.…”

Section: Patientmentioning

confidence: 99%

Amygdalae and Striatum Calcification in Lipoid Proteinosis

Gonçalves¹,

Melo²,

Matos³

et al. 2009

AJNR Am J Neuroradiol

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SUMMARY:Lipoid proteinosis is a rare genodermatosis characterized by multisystem involvement due to intracellular deposition of an amorphous hyaline material. Lipoid proteinosis is caused by mutations in the ECM1 gene. In many patients, skin and mucosa abnormalities are the first manifestation. When the CNS is affected, a wide variety of neurologic abnormalities may be present. The hallmark findings are calcifications, mostly occurring in the amygdalae, hippocampus, parahippocampal gyrus, or even the striatum. Present in half of the patients, moniliform blepharosis is considered a pathognomonic finding. In the other half of patients imaging could assist in the diagnosis. The authors present a series of 3 cases of lipoid proteinosis with brief clinical data and imaging findings.

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“…As the disease progresses to internal organs, an estimated 52% of cases have bilateral calcification of the medial temporal lobes (Emsley and Paster, 1985) resulting in considerable neuropsychiatric abnormalities (Thornton et al, 2008). In some of these cases, the bilateral calcification is restricted to the amygdala and even select subregions, providing unique insights into the role of the amygdala in both fear and panic processes.…”

Section: Introduction To Panic Disordermentioning

confidence: 99%

The Deakin/Graeff hypothesis: Focus on serotonergic inhibition of panic

Paul

Johnson

Shekhar

et al. 2014

Neuroscience & Biobehavioral Reviews

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The Deakin/Graeff hypothesis proposes that different subpopulations of serotonergic neurons through topographically organized projections to forebrain and brainstem structures modulate the response to acute and chronic stressors, and that dysfunction of these neurons increases vulnerability to affective and anxiety disorders, including Panic Disorder. We outline evidence supporting the existence of a serotonergic system originally discussed by Deakin/Graeff that is implicated in the inhibition of panic-like behavioral and physiological responses. Evidence supporting this panic inhibition system comes from the following observations: 1) serotonergic neurons located in the ‘ventrolateral dorsal raphe nucleus (DRVL) as well as the ventrolateral periaqueductal gray (VLPAG) inhibit dorsal periaqueductal gray-elicited panic-like responses; 2) chronic, but not acute, antidepressant treatment potentiates serotonin’s panicolytic effect; 3) contextual fear activates a central nucleus of the amygdala-DRVL/VLPAG circuit implicated in mediating freezing and inhibiting panic-like escape behaviors; 4) DRVL/VLPAG serotonergic neurons are central chemoreceptors and modulate the behavioral and cardiorespiratory response to panicogenic agents such as sodium lactate and CO2. Implications of the panic inhibition system are discussed.

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The Neuropsychiatry and Neuropsychology Of Lipoid Proteinosis

Cited by 55 publications

References 185 publications

Generous economic investments after basolateral amygdala damage

Generous economic investments after basolateral amygdala damage

Amygdalae and Striatum Calcification in Lipoid Proteinosis

The Deakin/Graeff hypothesis: Focus on serotonergic inhibition of panic

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