Marcello Mario Mascia

Combined EEG/fMRI recording in musicogenic epilepsy

Seizures induced by musical stimulation are usually correlated to temporal epilepsy, although the precise localization of their epileptogenic networks are not well characterized. Brain imaging studies show that regional cerebral blood flow (rCBF) recorded during musicogenic seizures is increased in putative epileptogenic foci, as well as in other brain regions not directly related to seizure activity. These studies, however, afford only a virtual temporal relation between ictal discharges and rCBF changes, given that brain images are correlated with off-line EEG recordings. To obviate this problem, the simultaneous multimodal recording of the episode of musicogenic epilepsy is mandatory. The present study describes the EEG-fMRI co-recording of musicogenic elicited seizures in a case of simple partial epilepsy. Our results show that EEG features recorded in epileptogenic areas are largely coupled with rCBF increase. Furthermore, rCBF modifications in other regions suggest that additional aspects of musical processing are also elicited during musicogenic seizures.

Bipolar affective disorder and Parkinson's disease: a rare, insidious and often unrecognized association

Abstract. Five patients (4 women) with Parkinsons disease (PD) and primary major psychiatric disorder (PMPD) meeting DSM-IV criteria for the diagnosis of bipolar affective disorder (BAD) were studied. Four patients had early onset PD. Four developed a severe psychiatric disorder a few years after starting dopaminergic therapy in presence of a mild motor disability and a mild cognitive impairment, with no evidence of cerebral atrophy at CT or MRI. Two patients developed a clear manic episode; the other three presented a severe depressive episode (in one case featuring a Cotard syndrome). None showed previous signs of long term L-dopa treatment syndrome (LTS), hallucinosis or other minor psychiatric disorders. The two manic episodes occurred shortly after an increase of dopaminergic therapy and in one case rapid cyclic mood fluctuations were observed. At the onset of psychiatric symptoms, all patients had an unspecific diagnosis of chronic delusional hallucinatory psychosis (CDHP).

Chronic delusional hallucinatory psychosis in early-onset Parkinson's disease: drug-induced complication or sign of an idiopathic psychiatric illness?

Abstract Chronic delusional psychosis with hallucinations (CDHP) is commonly assumed to complicate the later stages of Parkinsons disease, as a side effect of antiparkinsonian medication. We studied 7 patients with early onset PD, who had developed psychiatric manifestations consisting in CDHP after a few years of antiparkinsonian therapy. All patients underwent a neurological, psychiatric and brain imaging (CT or MRI) evaluation. Detailed clinical history was recorded in order to reveal prior psychiatric illness and to analyse the relationship between neurological disease, cognitive impairment and psychosis. Our findings suggest that CDHP occurring in patients with early onset PD, normal or slightly impaired cognitive functions and normal CT/MRI scans is invariably the expression of a coexisting psychiatric illness which prior to onset of the neurologic disease had not been correctly diagnosed and which has been disclosed by dopaminergic therapy.

Capgras syndrome in Parkinson’s disease: two new cases and literature review

The Capgras syndrome (CS) is a rare psychiatric disorder. CS is classified as a delusional misidentification syndrome. Initially, CS was described in paranoid schizophrenia and schizoaffective disorders. CS has also been reported in neurodegenerative diseases such as Alzheimer’s disease and Lewy body dementia. To date, there are very few descriptions of the occurrence of CS in idiopathic Parkinson’s disease (PD), with or without dementia. Considering the recent observation of two new cases in PD patients, a systematic overview of the literature published between 1976 and 2016 reporting CS in PD was conducted. The purpose of this article is to examine the phenomenon in people with PD with and without dementia, the psychopathologic context in which it happened, the role played by the dopaminergic medications and to define useful therapeutic strategies. Our CS cases occurred in two elderly patients with advanced PD and cognitive impairment, respectively, after an acute stressor event and after an increase of the total daily dose of levodopa. In light of our observations and the cases reported in the literature, we argue that CS is an acute or subacute psychotic disorder occurring mostly in PD with dementia. Besides, the increase in brain dopamine levels induced by acute stressful events and/or dopamine-enhancing medications should be considered as a possible causal mechanism of CS in patients with advanced stages of PD and cognitive decline.

Priapism and Hypersexuality Associated With Rotigotine in an Elderly Parkinsonian Patient: A Case Report.

To the Editor: P riapism is an uncommon condition defined as prolonged (greater than 4 hours) penile erection not initiated by sexual stimulation. There are many different causes of priapism including hematologic disorders, trauma, metabolic conditions, and medications. Several classes of medication are involved: antihypertensives, anticoagulants, α-blockers, antidepressants, psychoactive substances. In particular, about 50% of drug-induced priapism is due to typical and atypical antipsychotics, such as chlorpromazine, zuclopenthixol, clozapine, risperidone, olanzapine, quetiapine, ziprasidone,21 aripiprazole, and iloperidone. To date, there is only 1 case report on the association between priapism and cabergoline. Cabergoline is an ergotderived dopamine agonist with high affinity for the dopamine D2 receptor, but also possesses low affinity for dopamine D1, α1and α2-adrenergic, and 5-HT1and 5-HT2serotonin receptors. Herein we present the first detailed case of rotigotine-induced priapism and hypersexuality in a patient with Parkinson disease (PD). P.P. is a 79-year-old man, with a 10-year history of an idiopathic PD with resting tremor and rigidity more marked on the left side, bradykinesia, and camptocormia. Neuropsychological testing revealed subtle executive dysfunction and mild cognitive impairment. At the beginning, tremor, rigidity, and bradykinesia were well controlled under a treatment of a combination of levodopa/ carbidopa (400 mg/daily) and pramipexole extended release (1.05 mg/daily). Six years later, he experienced significant and unpredictable levodopa-induced motor fluctuations. At the time of our first observation, the patient showed the symptoms of an advanced and complicated PD with signs of fluctuating mood, sleep disturbances, such as excessive daytime sleepiness and falling asleep suddenly for short periods of time, similar to narcolepsy. Considering that the major cause of sleepiness was drug induced, we decided to reduce and then

Pisa-Like Syndrome Under Baclofen in a Patient With Spastic Hemiparesis due to Ischemic Stroke

In its original description, Pisa syndrome was reported as an iatrogenic dystonia of the trunk caused by neuroleptic drugs. However, sometimes, not dystonic lateral flexion of the trunk is described as Pisa syndrome. These observations support the possibility of a drug-induced lateral flexion of the trunk with clinical presentation similar to Pisa syndrome, although with a different etiology and pathophysiology. Here, we describe the case of a male patient, with a previous ischemic stroke and residual spastic hemiparesis to the right side, who subacutely developed a dramatic lateral flexion of trunk (approximately 45° to the right) a few days after the introduction of Baclofen (5 mg 3 times per day). After the discontinuation of baclofen, a full recovery of the correct posture was obtained. In this respect, our case is paradigmatic: it is drug-induced but not clearly dystonic in its manifestation. Baclofen reduces the spasticity depressing the monosinaptic and polisinaptic reflex in the spinal cord by stimulating Gamma-aminobutyric acid B (GABA-B) receptors, which inhibit the release of excitatory amino acids, glutamate and aspartate. We believe that the definition of Pisa syndrome for these forms, not clearly dystonic, might be not completely appropriate, but they should be defined more correctly as Pisa-like syndromes.

C9ORF72 Intermediate Repeat Expansion in a Patient With Psychiatric Disorders and Progressive Cerebellar Ataxia

Introduction: Large expansions of the noncoding GGGGCC repeat (more than 30) in the first intron of the C9ORF72 gene have been demonstrated to cause amyotrophic lateral sclerosis and frontotemporal dementia. Recent papers have investigated the possible pathogenic role and associated clinical phenotypes of hexanucleotide expansions with intermediate repeat lengths ranging between 20 and 29 repeats. Case Report: We report a case of a 71-year-old Sardinian female patient with a long history of psychiatric disorders such as mixed anxiety-depressive disorder associated with somatization disorder and histrionic personality who developed a slowly progressive cerebellar syndrome, mild cognitive impairment, pyramidal signs, and rapid eye movement sleep behavior disorder with imaging abnormalities on the DaTSCAN single-photon emission computed tomography indicating an alteration in the presynaptic dopaminergic system. The patient was found to have intermediate C9ORF72 repeat expansions. Conclusions: Early psychiatric presentations are a recurrent phenotypic manifestation of C9ORF72 expansions. In our patient, the intermediate C9ORF72 repeat expansion may have a pathogenic role in the cooccurrence of psychiatric and sleep disorders, cognitive dysfunctions, pyramidal system involvement, and late-onset cerebellar ataxia. This observation widens the spectrum of neurodegenerative conditions linked to C9ORF72 mutations.