Sirs: Dementia with Lewy bodies (DLB) is a frequent neurodegenerative dementing disorder [1]. In addition to cognitive impairments, features of DLB include psychiatric symptoms (e. g., visual hallucinations), parkinsonian features, fluctuations of cognition and consciousness, and increased sensitivity to neuroleptic drugs [2]. The most severe form of the neuroleptic sensitivity reaction is neuroleptic malignant syndrome (NMS) [3]. The incidence of NMS is substantially higher in DLB patients than in other dementias [4]. This observation suggests that brain sites which are crucial to the pathogenesis of NMS represent a specific component of the degenerative process in DLB. This hypothesis would be strongly supported if NMS occurred in DLB spontaneously. A 75-year-old woman with a three-year history of dementia including episodes of disorientation and confusion was admitted because of impairment of consciousness progressing to coma within 24 hours. Over the past 2–3 years, the patient had developed a gait disturbance (small steps, slowing of movements and stooped posture). Frequent unexplained falls were noted. On admission the comatose patient displayed marked generalized rigidity. Upon stimulation, posturing of extremities was noted. Intermittently, myoclonic jerks were observed. Plantar responses were upgoing bilaterally. Temperature was 40.2 °C, blood pressure 210/120 mmHg, pulse rate 130/min, and respiratory rate was 27/min. Excessive sweating was noted. The remainder of the medical examination was unremarkable. Two similar episodes had led to admissions in other institutions one month and one year earlier with insidiously developing “stupor”, hyperthermia, increased muscle tone, tachycardia, and markedly elevated serum levels of CK. These episodes had been fully reversible. Extensive enquiries did not reveal intake of neuroleptics or dopaminergic drugs or any other drug known to be related to NMS. CK on admission was elevated (118 U/l) and rose to 606 U/l on the following day. Infection, metabolic causes, epileptic seizures, intoxication, or cerebral ischemia were ruled out. Beta-amyloid 1–42 was decreased, while τ-protein was increased. Brain imaging studies revealed marked generalized atrophy and minimal signs of microangiopathy. Fluor-desoxyglucose positron emission tomography (FDG-PET) studies showed reduction of tracer uptake in the parieto-occipital region bilaterally and in the left basal ganglia, on top of a diffuse cerebral hypometabolism (Fig. 1a). Single photon emission computed tomography (SPECT) using I-[123]-Beta carbomethoxy-iodophenyltropane (CIT) and I-[123]-Iodobenzamid (IBZM) suggested reduced density of presynaptic dopamine carriers at the level of the basal ganglia bilaterally, and reduction of postsynaptic D2-dopamine receptors in the left basal ganglia, respectively (Fig. 1b, c). Following improvement over 14 days the patient suffered a spontaneous relapse with stupor, gaze deviation, increased muscle tone, myoclonic jerking and hyperthermia. From this, she recovered incompletely. This appears to be the first report of the spontaneous occurrence of an NMS-like condition in severe degenerative dementia. Although a final diagnosis cannot be made in the absence of neuropathology, findings strongly point to DLB as the underlying dementing disorder. Our patient exhibited a cognitive decline including episodes of confusion, and motor features of parkinsonism suggesting a diagnosis of “probable DLB” [2]. This diagnosis is further supported [2] by the symptoms “repeated falls” and “transient losses of consciousness”. In addition, FDG-PET showed parieto-occipital cerebral hypometabolism, in agreement with the specific pattern of DLB-patients found in imaging studies [5, 6] and at variance from the pattern associated with Alzheimer’s disease [5, 6]. Furthermore, PET imaging, IBZM-SPECTand 123-I-βCIT-SPECT-findings suggested basal ganglia hypometabolism and basal ganglia pre-and postsynaptic impairment of dopaminergic neurotransmission consistent with findings obtained in DLB-patients, but not in Alzheimer’s disease [7]. Decreased β-amyloid-levels [8] and increased τ-protein levels [9] are consistent with DLB. The syndrome of hyperthermia, rigidity, elevated CK and severely impaired consciousness with optional tachycardia, tachypnea, hypertension and diaphoresis has been named “neuroleptic malignant syndrome” although it may occur also in the absence of expoLETTER TO THE EDITORS
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Jun 1, 2012
Ronald F Pfeiffer 
Open Access