Journal of Sleep Disorders: Treatment and CareISSN: 2325-9639

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Case Report, J Sleep Disor Treat Care Vol: 1 Issue: 2

Togetherness of REM Behavior Disorder and Fahr Disease: A Case Report

Aylin Bican Demir*, Aygül Güneş, Damla Özyurtlu and İbrahim Bora
Department of Neurology, School of Medicine, Uludag University, Bursa, Turkey
Corresponding author : Aylin Bican Demir, MD
Department of Neurology, School of Medicine, Uludag University, Bursa, Turkey
Tel: +90 2242951723; Fax: +90 2244419177
E-mail: [email protected], [email protected]
Received: August 16, 2012 Accepted: September 12, 2012 Published: September 14, 2012
Citation: Demir AB, Günes A, Özyurtlu D, Bora I (2012) Togetherness of REM Behavior Disorder and Fahr Disease: A Case Report. J Sleep Disor: Treat Care 1:2. doi:10.4172/2325-9639.1000105

 

Abstract

Togetherness of REM Behavior Disorder and Fahr Disease: A Case Report

REM Behavior Disorder (RBD) is seen in elderly population mostly. Its prevalence is unknown but the disease was reported in 0.38% of overall population and in 0.50% of elderly population. One third of newly diagnosed Parkinson’s patients, 47% of overall Parkinson’s patients and 90% of multiple system atrophy patients diagnosed as RBD. RBD is a chronic, progressive sleep disorder and its frequency or severity changes over time. Fahr disease (FD) most common clinical signs are Parkinsonism, dystonia, tremor, chore, ataxia, dementia and affective disorders. Etiopathogenesis of RBD and FD are still unknown. Our patient presented with FD with the calcification in basal ganglions before Parkinson’s disease manifested. We aimed to underline the importance of FD in the differential diagnosis of RBD and RBD must be considered and kept in mind during the clinical course of FD.

Keywords:

Introduction

REM Behavior Disorder (RBD) is seen in elderly population mostly. Its prevalence is unknown but the disease was reported in 0.38% of overall population and in 0.50% of elderly population. One third of newly diagnosed Parkinson’s patients, 47% of overall Parkinson’s patients and 90% of multiple system atrophy patients diagnosed as RBD. RBD is a chronic, progressive sleep disorder and its frequency or severity changes over time [1,2].
Fahr disease (FD), also known as Bilateral Striopallidodentate calcinosis, was first defined in 1930. FD is a rare disease and its etiology is still unknown. Disease is characterized by calcification of nucleus dentatus of basal ganglion and deep cortical structures. Calcification may affect putamen, nucleus caudatus, internal capsule, nucleus dentatus, thalamus, cerebellum and vascular traces of cerebral white matter. Manifestation of symptoms reported mostly at 4th or 6th decades and rarely in pediatric patients [3,4]. Most common clinical signs are Parkinsonism, dystonia, tremor, chore, ataxia, dementia and affective disorders. Calcifications are mostly bilateral and might be seen with calcium-phosphor metabolism disorders. It has also been reported that some elements like calcium, iron, copper, various toxins and proteins can deposit and cause striopallidodentate calcifications after neuronal infectious diseases. Infectious diseases (Brucella, EBV, tuberculosis, AIDS), hyperthyroidism, hypothyroidism, tuberous sclerosis, SLE, motor neuron disorders are some of the diseases that take place in basal ganglion calcification etiology. Calcium deposits are mainly presents in capillary vessels, media layer and perivascular areas of small arteries and veins [3,5]. Deterioration mechanism of blood-brain barrier in Fahr disease is still unknown and it is not clear that whether it is secondary to deposition of calcium in blood-brain barrier or due to calcium metabolism disorder [3].

Case

A male patient born in 1961 was admitted to our emergency service with seizure in 2011 and was consulted to our department. In patient history his relatives mentioned that he had a seizure after falling down from a tree with eyes deviated to above and his chin locked for 2 minutes. After the seizure he had a period with nonsense speaking and random movements of limbs. Patient stated that he had no memory about that period and also mentioned that he sometimes had contractions in his right arm and leg lasting for 1 minute before this seizure manifested. The laboratory findings that assessed in ER was Ca:4.8 and cranial CT: Hyperintense appearance in bilateral basal ganglions and cerebellum (Fahr Disease- Figure 1). His neurological examination was normal and he was hospitalized for calcium infusion. His blood calcium level increased to 8.0 mg/ dl (Normal range: 8.4-10.2) and blood phosphor level was 5.8 mg/ dl (Normal range: 2.3-4.7) and parathormone level was 1.20 pg/ml (Normal range: 15-65). His full blood count, urine test, liver function, kidney function and thyroid function tests and EEG revealed as normal. Neuropsychometric tests resulted normal. The patient had calcium and vitamin D replacement and treatment continued with oral calcium therapy. Patient’s relatives mentioned that our patient had random movements and speaking behavior, mostly religious like praying, during sleep almost every night for several years. A polysomnography was performed (Figure 2) and some movements similar to religious praying ritual and speaking like praying was observed during REM phase of sleep. We started him on clonazepam with diagnosis of RBD and his symptoms significantly reduced with this therapy during hospital staying period and follow ups.
Figure 1: This picture, cranial CT is common in the calcification.
Figure 2: A- REM sleep stage, B-C-D- REM sleep stage, the patient starts to talk and is moving.

Discussion

RBD is a parasomnia characterized with complex motor activity associated with content of the dream due to absence of muscle atony which is normally seen during the REM phase of sleep. This phenomenon can be seen at all ages and both genders but it is mostly a disease of male gender and elderly people. Patients mostly become aware of the disease with disturbing the person that patient sleeps with, harming the patient itself or other problems that the dream caused [6]. RBD can be mistaken with other diseases and could be treated wrongly. The result with symptomatic treatment is mostly satisfying if the disease diagnosed correctly. Therefore RBD diagnosis is very important.
The incidence of the disease is 0.04-0.5% in overall population. It mostly effects male population above age of 50 and manifests mostly at age of 60s or 70s. Approximately 25% of patients had a prodromal phase characterized with speaking or partially extremity movements during REM phase of sleep. The median duration of this period is 22 years [1]. Our patient was 60 years old and had symptoms for several years like speaking during sleep and random extremity movements sometimes up to hitting to his wife. RBD exists in one third of newly diagnosed Parkinson’s disease patients and in 90% of patients with multiple system atrophy. RBD is not a simple parasomnia. The disease can be seen with sinucleopathies. Some multiple neurologic abnormalities like deterioration in cortical activity, decreased dopaminergic innervation, autonomic abnormalities and neuropsychological disorders might be seen with RBD [7]. Schenk et al. reported a case series consisting 96 RBD patients and they mentioned that 87.5% of the cases were old male patients [8].
Physiopathology of RBD is not clear. Jouvet et al. pointed out the relationship between atony during the REM phase of sleep and brainstem. This improvement has led to developing of new hypothesis about RBD physiopathology. These researchers demonstrated REM sleep without atony in cats with bilateral peri-locus coeruleus lesion [9]. Clinical studies about physiopathology of RBD revealed involvement of pedinculopontine nucleus, laterodorsal nucleus and nigrostriatal system [1,2,10,11]. Our case was diagnosed as Fahr Disease based on Cranial CT findings as hyperdense appearance of bilateral basal ganglions and cerebellum. Calcification findings in basal ganglions might been arose from Ca metabolism disorder and the same disorder may cause muscle contractions and a symptomatic seizure, there are reported cases about this phenomenon in the literature. RBD might have been developed due to an underlying calcium metabolism disorder. Reciprocal interaction model proposed by McCarley and Hobson revealed that REM sleep is initiated by cholinergic neurons and terminated by aminergic neurons [12].
RBD is frequently together with sinucleopathies like Parkinson’s disease, multisystem atrophy (MSA) and Lewy Body Dementia and might initiate several years before the diagnosis of these diseases were made. Our patient had an RBD clinic with absence of Parkinson’s or any other disorder. Possibility of neurodegenerative disorder development in RBD patients diagnosed at middle ages is very high. Subclinical RBD is very frequent even among Parkinson’s patients without sleeping symptoms. This phenomenon is ascribed to degenerative changes in pedunculopontine nucleus [13,14]. In a review consisting 280 RBD cases it was stated that 23% of patients had Parkinson’s disease [8]. 11 of 29 idiopathic RBD patients developed Parkinson’s or another similar disease in median 3.7 years. In another study consisting 39 multisystem atrophy patients; it was stated that 35 patient diagnosed as RBD after polysomnography and 27 patient had behavioral disorders associated with RBD. 12 of the patients had RBD diagnosis before MSA diagnosis was made [15].
Long term studies about RBD revealed that this disease is mostly concomitant with neurodegenerative disorders (Parkinson’s disease, MSA, Lewy body dementia) and other neurological diseases (SLE, brainstem tumors, Tourette Syndrome, Mabius Syndrome, ischemia of pons, subarachnoid bleeding, MS, Guillain-Barre Syndrome, mitochondrial encephalomyopathy, normal pressure hydrocephalus, Morvan’s Syndrome, voltage-gated potassium channel antibody associated limbic encephalitis, Juvenile Parkinson’s disease) are being discussed. RBD may be the first symptom of these disorders and could manifest decades before any symptom of neurodegenerative disorders [16,17]. Our patient presented with muscle contractions due to hypocalcaemia and clinical symptoms of RBD similar to other diseases.
Etiopathogenesis of RBD and Fahr diseases are still unknown. Our patient presented with Fahr disease with the calcification in basal ganglions before Parkinson’s disease manifested. We aimed to underline the importance of Fahr disease in the differential diagnosis of RBD and RBD must be considered and kept in mind during the clinical course of Fahr disease.

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