Neurocutaneous melanosis and psychosis: A case report

Neurocutaneous melanosis and psychosis: A case report
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  Psychiatry and Clinical Neurosciences (2001), 55 ,93–95 Regular Article Neurocutaneous melanosis and psychosis:A case report ANTONELLA AZZONI , md , 1 RAFFAELE ARGENTIERI,  md 2 ANDMICHELE RAJA,  md 1 1 Servizio Psichiatrico di Diagnosi e Cura,Ospedale Santo Spirito in Sassia and 2 Servizio di DermatologiaOspedale Nuovo Regina Margherita,Rome,Italy Abstract The paper describes a case of neurocutaneous melanosis (NM),with mental retardation,chronicpsychosis,and epilepsy possibly due to a temporal focus.This is the first report of NM associatedwith a severe and chronic psychosis.It is likely that such an association has not previously beendescribed because of the ominous prognosis of most cases of NM with early involvement of thecentral nervous system. Key words epilepsy,neurocutaneous melanosis,psychosis. INTRODUCTION Neurocutaneous melanosis (NM) is a rare congenitalsyndrome,possibly due to an error in the morphogen-esis of the embryonal neuroectoderm.It is character-ized by large or multiple congenital melanocytic neviand benign or malignant pigment cell tumors of theleptomeninges. 1 Most patients with NM present neu-rologic manifestations since early childhood,such asincreased intracranial pressure,mass lesions,or spinalcord compression. 2 Lower intelligence level and con-vulsive seizures often coexist. 3 In patients with a largecongenital melanocytic nevus (LCMN) and centralnervous system (CNS) involvement,the LCMN tendsto be present in a posterior axial location on thehead,neck,back,or buttocks and ‘satellite nevi’ areoften present. 4 Magnetic resonance imaging (MRI) of NM typically shows T1 shortening in the cerebellum,in the anterior temporal lobes and pia mater. 5 Even inthe absence of melanoma,the prognosis of NM isconsidered to be malignant. 2 CASE REPORT We present the case of a 44-year-old woman affectedby NM,with epilepsy,mental retardation and chronicpsychosis.The patient was admitted to our ward (S.Spirito Hospital,Rome,Italy) for behavioral disorder.She had no job,tended to vagrancy,and spent most of her time in institutions run by religious orders.Neu-ropsychiatric and dermatologic family history wasunremarkable.The patient had suffered from epilepsysince early childhood and was currently treated withphenobarbital and phenytoin.However,her compli-ance to treatment was poor and seizures were fre-quent.At that time,she suffered from complex partialand generalized tonic–clonic seizures.She presented aLCMN in a posterior axial location on the back andbuttocks (Fig.1) and hundreds of hyperpigmentedlesions on the face,trunk,and limbs.A giant edema of inferior limbs was present.On her visit,the patientwas alert and orientated.Neurologic and fundus oculiexamination was normal.She presented persecutorydelusions (she claimed that physicians from anotherhospital had poisoned her and had caused herepilepsy,and that her brother and her maid were plot-ting against her to take possession of her money) andreligious delusions (she considered her skin lesions tobe stigmata and it was her belief she had a holymission in life).Furthermore,there were presentauditory and visual hallucinations (Holy Spirit’s voiceand image),logorrhea,derailment,illogicality,andincoherence.She was repetitive and circumstantialand tended to hypergraphia.Persecutory and reli-gious delusions had been present for many years,withdevastating effects on her global functioning.Her per-sonality was characterized by dependence/passivityand hypermoralism.The Mini-Mental Status Exami-nation (MMSE) score was 23.Wechsler Adult Intelli-gence Score (WAIS) total IQ was 68 (verbal IQ 73;non-verbal IQ 66).Psychomotor coordination waspoor.Laboratory tests,echography of the heart,thyroid,abdomen,breast,and pelvis were unremark- Correspondence address:Michele Raja, MD ,via Prisciano 26,I-00136,Rome,Italy.Email:raja.russo@iol.itReceived 2 June 2000;revised 4 September 2000;accepted 19November 2000.  94A.Azzoni etal  . able.Electroencephalogram (EEG) revealed milddiffuse epileptiform signs (mono or biphasic spikesindependently occurring over both hemispheres,notprominent in the temporal lobes,and occasional sym-metric spike wave complexes).Contrast-enhancedbrain computed tomographic (CT) scan was normal.Unfortunately,MRI scanning was not available forthis patient.A skin biopsy of a pigmented lesionrevealed groups of melanocytes at the dermal–epider-mic junction and in the papillar derma and isles of nevic cells in the derma.This histologic architecturewas compatible with that of congenital nevi or so-called ‘deep penetrating nevi’.On day 9 of hospital-ization,the patient presented an absence-like seizure,followed an hour later by a generalized tonic–clonicseizure.The post-crisis EEG was similar to previousrecordings.The patient was treated with haloperidoldecanoate (100mg) and carbamazepine (up to a dailydose of 1600mg),with gradual tapering of phenytoinand phenobarbital and discharged with her psychi-atric symptoms improved.Reality test,communica-tion,and conceptual disorganization were muchimproved.Persecutory delusions and hallucinationswere no longer present,while bizarre religious ideaswere still present without influence on her behavior.At discharge,the EEG revealed occasional diffusesharp waves,without spikes or spike wave complexes.Unfortunately,the patient was lost to follow up.She began wandering again and refused medical controls. DISCUSSION The most probable diagnosis for this unusual case of neurocutaneous syndrome is NM.Skin biopsy,local-ization of LCMN,satellite nevi,and involvement of CNS (mental retardation,seizures and psychosis) cor-roborated the diagnosis.Neurocutaneous melanosis isoften accompanied by generalized or partial seizures. 6 In this patient,neurologic examination,EEG andbrain CT did not help to identify the epileptic focus(or foci) and,unfortunately,no other neuroimaging orneuro-physiologic studies were available.However,from a clinical perspective,it is possible to hypothe-size the presence of a temporal focus since the behav-ioral changes present in this patient are frequentlyobserved in patients with temporal lobe epilepsy(TLE): 7 1.A positive correlation has been found betweenpersistent interictal psychosis and epilepsy,in par-ticular TLE. 8 2.Hypergraphia,a tendency toward extensive oreven compulsive writing with preoccupation todetails,is present in approximately 8% of patientswith TLE. 9 3.Repetitive and circumstantial speech is one of themain traits of so-called viscosity,that has beenassociated with (especially left) TLE. 10 4.Hypermorality,hyperreligiosity,and dependenceare among the personality changes commonlyfound in patients affected by TLE. 11 Nevertheless,the lack of unequivocal localizingdata does not allow any firm conclusion to be madeabout the cerebral areas specifically involved in thebehavioral disorder of the present patient.Anotherunresolved issue is the pathophysiology of patient’spsychiatric symptoms.Three hypotheses are offered.First,psychiatric symptoms could be directly relatedto seizure activity and its sequelae.Second,corticalabnormalities due to ectoderm maldevelopment withaberrant neuronal migration could directly inducepsychiatric symptoms,as it happens in other neurode-velopmental disorders.The third,more specific,possi-bility is the excessive proliferation of melanotic cellsin the CNS,with subtle compression and dislocationor infiltration of brain structures.Although epilepsy and temporal lobe involvementare not rare in NM,and TLE is frequently associatedwith psychosis, 8 to our knowledge,this is the firstreport of NM associated with a severe and chronicpsychosis.It is likely that such an association has notbeen described previously because of the ominousprognosis of most cases of NM with early involve-ment of CNS.Contrary to most studies,but in accor-dance with the study of Martinez-Granero et al. , 3 thepresent case suggests that the survival prognosis for patients with neurologic involvement may befavorable. Figure1. Patient’s large congenital melanocytic nevus.  REFERENCES 1.Sasaki Y,Kobayashi S,Shimizu H,Nishikawa T.Multiple nodular lesions seen in a patient with neuro-cutaneous melanosis.  J. Dermatol. 1996;  23 :828–831.2.Kadonaga JN,Frieden IJ.Neurocutaneous melanosis:Definition and review of the literature.  J. Am. Acad.Dermatol. 1991;  24 :747–755.3.Martinez-Granero MA,Pascual-Castroviejo I,RocheHerrero MC,Fernandez-Jaen A,Calleja Perez B,Urgelles Fajardo E.Neurocutaneous melanosis andcongenital melanocytic nevi:Report of 6 cases.  Neurolo- gia 1997;  12 :287–292.4.DeDavid M,Orlow SJ,Provost N  etal. Neurocutaneousmelanosis:clinical features of large congenitalmelanocytic nevi in patients with manifest centralnervous system melanosis.  J. Am. Acad. Dermatol. 1996; 35 :529–538.5.Barkovich AJ,Frieden IJ,Williams ML.MR of neurocu-taneous melanosis.  Am. J. Neuroradiol. 1994;  15 :859–867.6.Pascual-Castroviejo I.Neurocutaneous melanosis.  In :Gomez MR (ed.).  Neurocutaneous Diseases:A Practical  Approach. Butterworths,Boston,1987;329–334.7.Paraiso J,Devinsky O.Neurobehavioral aspects of epilepsy.  In :Feinberg TE,Farah MJ (eds).  Behavioral Neurology and Neuropsychology. McGraw-Hill,NewYork,1997;641–656.8.Perez M,Trimble MR.Epileptic psychosis – Diagnosticcomparison with process schizophrenia.  Br. J. Psychiatry 1980;  137 :245–249.9.Sachdev HS,Waxman SG.Frequency of hypergraphia intemporal lobe epilepsy:An index of interictal behaviorsyndrome.  J. Neurol. Neurosurg. Psychiatry 1981;  44 :358–360.10.Brandt J,Seidman LJ,Kohl D.Personality characteri-stics of epileptic patients:A controlled study of general-ized and temporal lobe cases.  J. Clin. Experim.Neuropsychology 1985;  7 :25–38.11.Bear DM,Fedio P.Quantitative analysis of interictalbehavior in temporal lobe epilepsy.  Arch. Neurology 1977;  34 :454–467.Neurocutaneous melanosis and psychosis95
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