Progressive and accelerated weight and body fat loss in Parkinson's disease: A three-year prospective longitudinal study

IntroductionAlthough weight loss is common in Parkinson's disease (PD), longitudinal studies assessing weight and body composition changes are limited.MethodsIn this three-year longitudinal study, 125 subjects (77 PD patients and 48 spousal/sibling controls) underwent clinical, biochemical and body composition assessments using dual-energy X-ray absorptiometry.ResultsPatients were older than controls (65.6 ± 8.9 vs. 62.6 ± 7.1, P = 0.049), with no significant differences in gender, comorbidities, dietary intake and physical activity. Clinically significant weight loss (≥5% from baseline weight) was recorded in 41.6% of patients, with a doubling of cases (6.5 to 13.0%) classified as underweight at study end. Over three years, patients demonstrated greater reductions in BMI (mean −1.2 kg/m², 95%CI-2.0 to −0.4), whole-body fat percentage (−2.5% points, 95%CI-3.9 to −1.0), fat mass index (FMI) (−0.9 kg/m², 95%CI-1.4 to −0.4), visceral fat mass (−0.1 kg, 95%CI-0.2 to 0.0), and subcutaneous fat mass (−1.9 kg, 95%CI-3.4 to −0.5) than in controls, with significant group-by-time interactions after adjusting for age and gender. Notably, 31.2% and 53.3% of patients had FMI<3rd (severe fat deficit) and <10th centiles, respectively. Muscle mass indices decreased over time in both groups, without significant group-by-time interactions. Multiple linear regression models showed that loss of body weight and fat mass in patients were associated with age, dyskinesia, psychosis and constipation.ConclusionsWe found progressive loss of weight in PD patients, with greater loss of both visceral and subcutaneous fat, but not muscle, compared to controls. Several associated factors (motor and non-motor disease features) were identified for these changes, providing insights on possible mechanisms and therapeutic targets.     

Progressive rubella panencephalitis. Follow-up EEG study of a case.

Progressive rubella panencephalitis is a very rare slow virus disease of the nervous system. The authors present a case, concerning a young man, aged 20 years, died 11 months after the onset of the disease. The following peculiarities of the case are emphasized: 1) the clinical symptomatology and the evolution (myoclonus, lack of cerebellar impairment) could suggest the diagnosis of SSPE; 2) the EEG recordings showed epileptiform abnormalities, long latency diffuse periodic complexes and--during interferon therapy and simultaneously with a temporary clinical improvement--the appearance of short latency anterior periodic complexes.     

Progressive loss of raphe nuclei serotonin transporter in early Parkinson's disease: A longitudinal 123I-FP-CIT SPECT study

BackgroundSerotonergic raphe nuclei dysfunction has been documented in Parkinson's disease, both in pathological and neuroimaging studies, and has been associated with scores of tremor and non-motor symptoms. However, no in vivo longitudinal investigations have been conducted to assess the rate of decline of raphe serotonin transporter availability in the early stages of the disease.ObjectiveTo measure the rate of decline of raphe serotonin transporter availability over a two-year interval in patients with recently diagnosed disease and its association with non-motor symptoms over time.MethodsBaseline and two-year follow-up ¹²³ioflupane-fluoropropyl-carbomethoxy-3-beta-4-iodo-phenyltropane (¹²³I-FP-CIT) SPECT scans of 173 early Parkinson's disease patients enrolled in the Parkinson's Progressive Markers Initiative were analysed and non-motor symptoms scores recorded.ResultsA 16.6 ± 20.9% (mean ± SD) reduction in raphe serotonin transporter availability was found from baseline to two-year follow-up in the entire cohort. No differences in progression were found between tremor dominant and postural instability/gait difficulty phenotypes. At follow-up 34.1% of patients showed a moderate-to-severe reduction of raphe serotonin transporter availability with respect to the controls’ mean. We did not find any significant correlation between raphe serotonin transporter availability and scores of depression, excessive daytime sleepiness and REM sleep behaviour disorder.Conclusion¹²³I-FP-CIT SPECT was able to measure longitudinal reductions in raphe serotonin transporter availability in the early phases of Parkinson's disease. About four years after diagnosis, raphe serotonin transporter availability was significantly reduced in more than one third of the population, but does not appear to be correlated to non-motor symptoms at this stage.     

Oculomotor behaviour in simultanagnosia: a longitudinal case study

The aim of the present single case study was to investigate oculomotor recovery in a patient with simultanagnosia due to biparietal hypoxic lesions. Applying visual exploration as well as basic oculomotor tasks in three consecutive test sessions--i.e. 8 weeks, 14 weeks, and 37 weeks after brain damage had occurred--differential recovery was observed. While visual exploration remarkably improved, an impaired disengagement of attention persisted. The improvement of exploration behaviour is interpreted within an oculomotor network theory and implications for a deficit-specific recovery from simultanagnosia are discussed.     

Progressive visual loss due to a muslinoma--report of a case and review of the literature.

Intracranial internal carotid artery (ICA) aneurysms are frequently treated either by microsurgical clipping of the aneurysm neck, by endovascular coiling of the aneurysm sac or by balloon occlusion of the parent vessel. For some broad-based aneurysms that may not be suitable for any of these options, microsurgical wrapping of the aneurysm wall with muslin or gauze rarely is applied. We report the case of a patient who suffered from a minor stroke because of arterio-arterial embolism from an intracranial ICA aneurysm. The aneurysm was treated by wrapping muslin material. After 12 months, he experienced progressive visual loss. Cranial magnetic resonance testing magnetic resonance tomography (MRT) revealed a granulomatous inflammation surrounding the site of the aneurysm and affecting optochiasmatic structures. A muslin-induced optic neuropathy is a rare but serious complication of a chronic inflammatory reaction in response to muslin or gauze used in intracranial aneurysm wrapping. If the foreign body inflammatory reaction to muslin or gauze leads to a mass formation, the term muslinoma or gauzoma is applied. Various treatment strategies include surgery, steroids and cyclophosphamide, but recovery of the vision is not predictable. As a consequence, muslin or cotton gauze should only be applied with great caution in neurovascular surgery.     

Progressive leptomeningeal fibrosis: a clinico-pathological case report.

A female patient developed persistent facial pain beginning at age 19 years. Intermittent motor and sensory disturbances referable to one hemisphere began nine years later and by the age of 41 she had developed signs of increased intracranial pressure. Exploratory craniotomy revealed replacement of the leptomeninges by thick, fibrous tissue. The histological appearance was that of a chronic, benign and minimally infiltrative process with a mild, non-specific inflammatory component, underlying cortical ischaemic changes, and white matter oedema. The lesion resembled nodular fasciitis, a soft tissue process. No cause of the reactive fibrosis of the meninges in this case is known.     

Neurodevelopmental outcome for extended cold water drowning: a longitudinal case study.

There is little longitudinal data examining outcome of pediatric near-drowning. Most literature tracks status 5 years or less post insult, focusing primarily on gross neurologic status as opposed to more subtle neurocognitive deficits. The present case tracks the neuropsychological profile of a child who was submerged for 66 min, the longest time documented. Acute medical support was aggressive, and recovery was dramatic, being featured in multiple media reports. Although an article published 6 years after the near-drowning described the child as "recovering completely," the longitudinal profile indicates a pronounced pattern of broad cognitive difficulties, particularly notable for global memory impairment. Neuropsychological test results were significant despite the fact that the patient's recent MRI and MEG were within normal limits. This case demonstrates the need for long-term neuropsychological follow-up of pediatric patients with histories of neurologic injury, as gross neurological examination and MRI and MEG scans may not reveal underlying brain dysfunction.     

Progressive unilateral hypertrophic myopathy: A case study

A 12-year-old girl had progressive unilateral muscle hypertrophy limited to the sole, tibialis anterior, and biceps femoris muscles. The affected muscles showed complex repetitive discharges by electromyography, necrosis and variation of fiber size upon histopathological examination, and increased metabolic activity in biochemical studies. The findings suggest a myopathic origin, but the actual cause remains unknown. © 1993 John Wiley & Sons, Inc.     

Progressive nonfluent aphasia with dementia: a case report.

We report a patient with progressive nonfluent aphasia and mild dementia. On 9-month follow-up evaluation, there was significant deterioration in both language and cognition. Unlike in Alzheimer's disease, the aphasia was of the Broca's type. In contrast with primary progressive aphasia, a dementia was present and the cognitive and language decline showed a rather rapid course.     

Progressive spinal muscular atrophy and parathyroid adenoma. Clinico-pathologic study of a case

A 82 year-old man died 6 years after the onset of a progressive spinal muscular atrophy. Post-mortem examination disclosed a parathyroid adenoma. Weakness and wasting were prominent in the proximal lower limbs. There were no fasciculations. Involvement of the medulla was mild and late. These clinical features were also present in 16 reported cases, which were improved by treatment of primary or secondary hyperparathyroidism. Our patient differs by the involvement of the hand muscles and the loss of tendon reflexes. Neuropathological study, as in one other reported case, showed a loss of anterior horn cells. Such cases underline that calcium metabolism must be studied in syndromes of spinal muscular atrophy.     

Progressive multifocal leukoencephalopathy: a burnt-out case

A patient with Hodgkin's disease developed progressive multifocal leukoencephalopathy (PML), documented by brain biopsy to be associated with JC virus infection. His disease progressed over several months, resulting in severe neurological deficit, but then stabilized with little or no further clinical progression during the remaining year of his life. Histopathological evaluation of the brain at autopsy supported the clinical impression that brain infection was arrested. Whereas the brain biopsy exhibited the histological features of active PML including giant bizarre astrocytes, at postmortem examination brain lesions appeared inactive, with regression of astrocytic changes and elimination of oligodendroglial inclusions. Similarly, JC virus antigen, present in the brain biopsy, was not detected in the autopsied brain. This case provides further evidence that PML is not invariably fatal and that clinical and cytological remission can occur.     

Phenol reduces hypertonia and enhances strength: a longitudinal case study

Phenyl alcohol blocks are used to relieve spasticity. Such nerve conduction blocks result from phenol-induced axonotmesis and could potentially affect muscle properties related to the ability to generate, maximize, and reduce force. This study assessed the 12-week longitudinal effect of phenol on position (stiffness) and velocity (damping) components of hypertonia, in addition to strength (peak torque and times to generate and reduce torque) in an individual with chronic elbow flexor spasticity following stroke. Phenol motor point injections of flexor muscles paradoxically increased the magnitude of flexion torque and decreased the times required to generate and reduce flexion and extension joint torques, in addition to reducing elbow extension stiffness and damping. Large reductions in the velocity-related component of hypertonia (damping changes > 90%) occurred immediately following injection, which is a finding that supports the velocity-dependent definition of spasticity. Although the changes in damping were large and transient, changes in stiffness and strength variables were small, slower to occur, and maintained. This suggests secondary changes following nerve block, possibly facilitated by regular elbow use subsequent to spasticity reduction.     

Progressive anarthria with secondary parkinsonism: a clinico-pathological case report.

The pathological process and lesion topography in patients with the syndrome of progressive aphasia are heterogeneous and few necropsy examination cases have been investigated. This is a case report of a 53 year old right handed man with progressive anarthria and secondary Parkinsonism over a period of six years. Positron emission tomography (PET) showed a decreased cerebral blood flow and metabolism in the frontal cortex, which was more pronounced on the left. Neuropathology disclosed a spongiform vacuolation in layer II of the frontal cortex, mostly in the Broca area, and neuronal loss in the substantia nigra. This original case reinforces the view that there are different entities of the syndrome of progressive aphasia which can be identified on the basis of clinical, neuroimaging and anatomical data.     

Visual memory loss and autobiographical amnesia: a case study

Amnesia typically results from trauma to the medial temporal regions that coordinate activation among the disparate areas of cortex that represent the information that make up autobiographical memories. We proposed that amnesia should also result from damage to these regions, particularly regions that subserve long-term visual memory [Rubin, D. C., & Greenberg, D. L. (1998). Visual memory-deficit amnesia: A distinct amnesic presentation and etiology. Proceedings of the National Academy of Sciences of the USA, 95, 5413-5416]. We previously found 11 such cases in the literature, and all 11 had amnesia. We now present a detailed investigation of one of these patients. M.S. suffers from long-term visual memory loss along with some semantic deficits; he also manifests a severe retrograde amnesia and moderate anterograde amnesia. The presentation of his amnesia differs from that of the typical medial-temporal or lateral-temporal amnesic; we suggest that his visual deficits may be contributing to his autobiographical amnesia.     

Periodic psychosis of puberty. A longitudinal case study.

A number of reports indicate that many women experience minor physical and psychological symptoms just prior to or during menses. Rarer episodic psychotic states associated with menses have received scanty attention. In the few available case reports, many hypotheses about their etiology have been advanced, usually unsupported by systemic studies. A 15-year-old girl with periodic psychotic episodes associated with menses was studied over many months. The interval between her periods was around 45 days. Psychotic episodes usually started a few days before or in association with menstural bleeding. Typically characterized by withdrawal, negativism, an apprehensive attitude, and suspiciousness, they remitted fully in 10 to 12 days with or without treatment. To facilitate necessary behavioral observations and complete various investigations, the patient was kept for nearly 60 days without treatment as an inpatient. Repeated vaginal smear cytology examinations during the menstrual cycle indicated a predominant estrogen influence throughout the cycle with an absence of the expected progesterone predominance in its later part. The patient gained 5 pounds between two cycles. Therapy with progesterone was instituted following the observation period. This patient's Rorschach revealed strong conflicts in relation to her sexuality. Concomitant with change in vaginal cytology reflecting a more balanced estrogen/progesterone influence, the patient stopped having psychotic episodes with menses. During a trial period when progesterone therapy was stopped, the psychotic episodes recurred but were prevented with its subsequent reinstitution. A complex interplay of hormonal factors that brings heightened sexual drive and possibly water retention in a predisposed patient can contribute to the causation of psychotic episodes at menstrual onset. Menstrual bleeding can associatively facilitate sexual fantasies and heighten conflict. Under this combined stress, the ego's defensive structure is likely to crumble with resultant psychotic episodes.     

Motor evoked potentials in a case of stiff-man syndrome: a longitudinal study

The clinical and neurophysiological findings in a patient with a typical stiff-man syndrome and their three-year evolution are described. The patient had high titers of anti-glutamic acid decarboxylase antibodies in both serum and cerebrospinal fluid. Magnetic resonance imaging (MRI) of brain and spinal cord was normal. Transcranial magnetic stimulation (TMS) revealed a distinctive motor evoked potential (MEP) pattern in proximal lower limb muscles consisting of markedly increased MEP amplitudes and MEP/M ratios, reduced excitability thresholds, and absent silent period. However, MEP latencies, central and peripheral conduction times and amplitudes obtained by magnetic spinal root stimulation were normal. Treatment with benzodiazepine and baclofen normalized both the clinical picture and the MEP values. TMS may be useful both as a diagnostic tool and to monitor the response to drug treatment.