Neuropsychological Profile of Internal Frontal Hyperostosis

Abstract

AbstractBackgroundIn 1765 Giovanni Morgagni described internal frontal hyperostosis syndrome (IFH), obesity and hirsutism. Stevart (1928) and Morel (1930) added the presence of neurological and neuropsychiatric involvement, constituting the definition of Morgagni‐Stewart‐Morel Syndrome (MSM). There are cases of HFI with absence of the rest of symptoms.MethodClinical, neuropsychological and neuropsychiatric evaluation of 5 patients treated for cognitive and behavioral impairment in whom neuroimaging neuroimaging showed internal frontal hyperostosis, apparently of sufficient entity to be associated with frontal lobe compression. Independent evaluation of the rest of the clinical and and family history that could indicate the presence of the constellation of constitutive symptoms of MSM.ResultThe neuropsychological battery showed as fundamental findings: depressive mood, attentional deficit, deterioration of working and recent memory, loss of concentration. No productive psychotic symptoms. Difficulty in complex tasks or complex skills. Anhedonia. No obsessive or compulsive symptomatology, nor anxiety as a predominant symptom. Irritability and irascibility. Mnesic process of inactive profile, difficulty in the change of mnesic strategy or language, or inhibition of irrelevant stimuli, problems with calculation, reasoning, sequencing or mental organization. Reduced verbal and non‐verbal fluency and disordered self‐regulation. Attention was drawn to the discordance between the significant functional impairment of the patients and the relatively preserved performance on neurocognitive battery.ConclusionThe presence of psychiatric and neurocognitive symptomatology associated with HFI has a characteristic profile, a varied constellation of deficits attributable to frontal lobe dysfunction, but should be actively investigated, because of its difficult until it significantly affects the patient’s performance.