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Du F, Zheng JW, Zhao YB, Yang K, Li HN. Full neurological recovery from severe nonexertional heat stroke with multiple organ dysfunction: A case report. World J Clin Cases 2023; 11:2355-2362. [PMID: 37122509 PMCID: PMC10131031 DOI: 10.12998/wjcc.v11.i10.2355] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/26/2022] [Revised: 02/05/2023] [Accepted: 03/09/2023] [Indexed: 05/02/2023] Open
Abstract
BACKGROUND We report a rare case of full neurological recovery from severe nonexertional heat stroke in a 67-year-old woman with an initial Glasgow Coma Scale of 3. This report raises awareness among doctors that when heatstroke is diagnosed, comprehensive treatment should be implemented as soon as possible. Moreover, targeted temperature management, combination therapy with hemodialysis and hemoperfusion, and hyperbaric oxygen therapy may alleviate multiorgan failure and prevent neurological sequelae caused by heatstroke.
CASE SUMMARY A previously healthy 67-year-old woman with an initial Glasgow Coma Scale of 3 was found lying prone on the road at noon on a summer day. Laboratory tests revealed multiorgan failure. As soon as heatstroke was diagnosed, comprehensive treatment was implemented. On hospital Day 3, the patient was extubated. Her initial Sequential Organ Failure Assessment score at hospitalization was 14 and decreased to 2 on hospital Day 4. On the seventh day following hospital admission, as the patient’s general condition improved, the levels of laboratory test findings decreased rapidly. Finally, the patient gradually recovered with no other neurological symptoms (the Glasgow Coma Scale at discharge was 15, and her ability to walk independently was restored).
CONCLUSION This case demonstrated that targeted temperature management, combination therapy with hemodialysis and hemoperfusion, and hyperbaric oxygen therapy may alleviate multiorgan failure and prevent neurological sequelae caused by heatstroke.
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Affiliation(s)
- Fang Du
- Emergency and Critical Care Center, Hubei University of Medicine, Renmin Hospital, Shiyan 442000, Hubei Province, China
| | - Jun-Wei Zheng
- Department of Anesthesiology, Hubei University of Medicine, Renmin Hospital, Shiyan 442000, Hubei Province, China
| | - Yan-Bo Zhao
- Emergency and Critical Care Center, Hubei University of Medicine, Renmin Hospital, Shiyan 442000, Hubei Province, China
| | - Kai Yang
- Emergency and Critical Care Center, Hubei University of Medicine, Renmin Hospital, Shiyan 442000, Hubei Province, China
| | - Hu-Nian Li
- Emergency and Critical Care Center, Hubei University of Medicine, Renmin Hospital, Shiyan 442000, Hubei Province, China
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2
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Yu T, Korgaonkar MS, Grieve SM. Gray Matter Atrophy in the Cerebellum-Evidence of Increased Vulnerability of the Crus and Vermis with Advancing Age. THE CEREBELLUM 2017; 16:388-397. [PMID: 27395405 DOI: 10.1007/s12311-016-0813-x] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/28/2022]
Abstract
This study examined patterns of cerebellar volumetric gray matter (GM) loss across the adult lifespan in a large cross-sectional sample. Four hundred and seventy-nine healthy participants (age range: 7-86 years) were drawn from the Brain Resource International Database who provided T1-weighted MRI scans. The spatially unbiased infratentorial template (SUIT) toolbox in SPM8 was used for normalisation of the cerebellum structures. Global volumetric and voxel-based morphometry analyses were performed to evaluate age-associated trends and gender-specific age-patterns. Global cerebellar GM shows a cross-sectional reduction with advancing age of 2.5 % per decade-approximately half the rate seen in the whole brain. The male cerebellum is larger with a lower percentage of GM, however, after controlling for total brain volume, no gender difference was detected. Analysis of age-related changes in GM volume revealed large bilateral clusters involving the vermis and cerebellar crus where regional loss occurred at nearly twice the average cerebellar rate. No gender-specific patterns were detected. These data confirm that regionally specific GM loss occurs in the cerebellum with age, and form a solid base for further investigation to find functional correlates for this global and focal loss.
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Affiliation(s)
- Teresa Yu
- The Brain Dynamics Centre, Westmead Millennium Institute and Sydney Medical School, Sydney, NSW, Australia
| | - Mayuresh S Korgaonkar
- The Brain Dynamics Centre, Westmead Millennium Institute and Sydney Medical School, Sydney, NSW, Australia.,Discipline of Psychiatry, Sydney Medical School, The University of Sydney, Westmead Hospital, Sydney, NSW, Australia.,Sydney Translational Imaging Laboratory, Heart Research Institute, Charles Perkins Centre and Sydney Medical School, University of Sydney, Sydney, NSW, 2006, Australia
| | - Stuart M Grieve
- The Brain Dynamics Centre, Westmead Millennium Institute and Sydney Medical School, Sydney, NSW, Australia. .,Sydney Translational Imaging Laboratory, Heart Research Institute, Charles Perkins Centre and Sydney Medical School, University of Sydney, Sydney, NSW, 2006, Australia. .,Department of Radiology, Royal Prince Alfred Hospital, Camperdown, Sydney, NSW, 2006, Australia.
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3
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Moon HJ, Jeon B. Can Therapeutic-Range Chronic Phenytoin Administration Cause Cerebellar Ataxia? J Epilepsy Res 2017; 7:21-24. [PMID: 28775951 PMCID: PMC5540686 DOI: 10.14581/jer.17004] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/17/2017] [Accepted: 03/13/2017] [Indexed: 11/21/2022] Open
Abstract
Phenytoin (PHT) is a first line antiepileptic drug (AED) used to treat many epilepsy syndromes. As with other AEDs, there are various adverse effects associated with PHT. For this brief review, we searched for evidence of cerebellar ataxia as a chronic adverse effect of therapeutic-range PHT treatment. Many previous studies appeared related to this issue, but many of those studies were designed to resolve questions related to the persistent residual effects of toxic-range PHT therapy, or they were inconclusive due to an absence of critical information such as PHT serum concentration, cerebellar symptoms/signs, and other factors contributing to cerebellar degeneration. Nevertheless, there were a few reports suggesting that cerebellar ataxia may be a chronic adverse effect of therapeutic-range PHT therapy and that a possible pathomechanism for that effect is folate deficiency. Moreover, there is the possibility that there may be patient-specific susceptibility factors affecting ataxia. Further studies are needed to elucidate the incidence, risk factors, and pathomechanism of cerebellar ataxia as a chronic adverse effect of therapeutic-range PHT treatment.
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Affiliation(s)
- Hye-Jin Moon
- Department of Neurology, Keimyung University Dongsan Medical Center, Daegu, Korea
| | - Beomseok Jeon
- Department of Neurology and Movement Disorder Center, Seoul National University Hospital, Seoul, Korea
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4
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Hayakawa K, Kumagai H, Suzuki Y, Furusawa N, Haga T, Hoshi T, Fujiwara Y, Yamaguchi K. Mr Imaging of Chronic Alcoholism. Acta Radiol 2016. [DOI: 10.1177/028418519203300302] [Citation(s) in RCA: 25] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
We evaluated the brain lesions of patients with chronic alcoholism (n = 34) in comparison with age- and sex-matched controls (n = 40) by MR imaging. T1-weighted sagittal and axial images and T2-weighted axial images were obtained with a 0.5 T superconducting MR unit. Various brain measurements were then performed, and the presence of regions of abnormal signal intensity was also compared between the two groups. The brain measurements revealed significant cerebral atrophy (characterized by lateral and 3rd ventricular dilatation, and widening of the interhemispheric fissure) as well as significant cerebellar atrophy (represented by 4th ventricular dilatation) in the alcoholic group. These changes were more prominent in patients in their fifties and sixties than in those aged in the thirties and forties. Focal hypointense lesions were observed in 20.6% of the alcoholics and in 5% of the controls (p < 0.01), while focal hyperintense lesions were observed in 61.8% of the alcoholics and in 20% of the controls (p < 0.001). The severity of these MR findings correlated well with the age of the patients. These observations suggest that alcohol is an important promotor of brain aging.
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5
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Calton MA, Howard JR, Harper RM, Goldowitz D, Mittleman G. The Cerebellum and SIDS: Disordered Breathing in a Mouse Model of Developmental Cerebellar Purkinje Cell Loss during Recovery from Hypercarbia. Front Neurol 2016; 7:78. [PMID: 27242661 PMCID: PMC4865515 DOI: 10.3389/fneur.2016.00078] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/03/2016] [Accepted: 05/02/2016] [Indexed: 12/05/2022] Open
Abstract
The cerebellum assists coordination of somatomotor, respiratory, and autonomic actions. Purkinje cell alterations or loss appear in sudden infant death and sudden death in epilepsy victims, possibly contributing to the fatal event. We evaluated breathing patterns in 12 wild-type (WT) and Lurcher mutant mice with 100% developmental cerebellar Purkinje cell loss under baseline (room air), and recovery from hypercapnia, a concern in sudden death events. Six mutant and six WT mice were exposed to 4-min blocks of increasing CO2 (2, 4, 6, and 8%), separated by 4-min recovery intervals in room air. Breath-by-breath patterns, including depth of breathing and end-expiratory pause (EEP) durations during recovery, were recorded. No baseline genotypic differences emerged. However, during recovery, EEP durations significantly lengthened in mutants, compared to WT mice, following the relatively low levels of CO2 exposure. Additionally, mutant mice exhibited signs of post-sigh disordered breathing during recovery following each exposure. Developmental cerebellar Purkinje cell loss significantly affects compensatory breathing patterns following mild CO2 exposure, possibly by inhibiting recovery from elevated CO2. These data implicate cerebellar Purkinje cells in the ability to recover from hypercarbia, suggesting that neuropathologic changes or loss of these cells contribute to inadequate ventilatory recovery to increased environmental CO2. Multiple disorders, including sudden infant death syndrome (SIDS) and sudden unexpected death in epilepsy (SUDEP), appear to involve both cardiorespiratory failure and loss or injury to cerebellar Purkinje cells; the findings support the concept that such neuropathology may precede and exert a prominent role in these fatal events.
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Affiliation(s)
- Michele A Calton
- Department of Psychology, The University of Memphis , Memphis, TN , USA
| | - Jeremy R Howard
- Department of Psychology, The University of Memphis , Memphis, TN , USA
| | - Ronald M Harper
- Neurobiology, David Geffen School of Medicine, University of California, Los Angeles , Los Angeles, CA , USA
| | - Dan Goldowitz
- Centre for Molecular Medicine and Therapeutics, University of British Columbia , Vancouver, BC , Canada
| | - Guy Mittleman
- Department of Psychological Science, Ball State University , Muncie, IN , USA
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6
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Dubbioso R, Pellegrino G, Antenora A, De Michele G, Filla A, Santoro L, Manganelli F. The Effect of Cerebellar Degeneration on Human Sensori-motor Plasticity. Brain Stimul 2015; 8:1144-50. [DOI: 10.1016/j.brs.2015.05.012] [Citation(s) in RCA: 29] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/22/2015] [Revised: 04/20/2015] [Accepted: 05/30/2015] [Indexed: 10/23/2022] Open
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7
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Mavroudis IA, Manani MG, Petrides F, Kiourexidou M, Njau SN, Costa VG, Baloyannis SJ. Dendritic, axonal, and spinal pathology of the Purkinje cells and the neurons of the dentate nucleus after long-term phenytoin administration: a case report. J Child Neurol 2013; 28:1299-304. [PMID: 22965563 DOI: 10.1177/0883073812455694] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
Phenytoin is a commonly prescribed anticonvulsant drug; however, there is evidence that long-term administration is related to cerebellar ataxia, cerebellar atrophy, loss of Purkinje cells, and hyperplasia of Bergman glia cells. The aim of the present study was to detect and describe any possible alterations of the Purkinje cells, and neurons of the dentate nucleus, as those can be seen with the use of silver impregnation techniques, such as Golgi and Nauta method. The study was performed on a 7-year-old boy who was under phenytoin treatment for more than 3.5 years and had clinical manifestations of cerebellar ataxia. Golgi silver impregnation technique revealed substantial loss of dendritic spines and tertiary dendritic branches, both on the Purkinje cells and the neurons of the dentate nucleus, whereas the Nauta method demonstrated swollen and degenerated axons of Purkinje cells.
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Affiliation(s)
- Ioannis A Mavroudis
- 1Department of Neurology, Laboratory of Neuropathology and Electron Microscopy, Aristotle University of Thessaloniki, Greece
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8
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Corti M, Villafañe MF, Palmieri O. [Cerebellar atrophy in a HIV/AIDS patient]. Rev Clin Esp 2007; 207:103-4. [PMID: 17397579 DOI: 10.1016/s0014-2565(07)73326-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/21/2022]
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9
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Maschke M, Weber J, Bonnet U, Dimitrova A, Bohrenkämper J, Sturm S, Müller BW, Gastpar M, Diener HC, Forsting M, Timmann D. Vermal atrophy of alcoholics correlate with serum thiamine levels but not with dentate iron concentrations as estimated by MRI. J Neurol 2005; 252:704-11. [PMID: 15778906 DOI: 10.1007/s00415-005-0722-2] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/27/2004] [Revised: 10/20/2004] [Accepted: 11/04/2004] [Indexed: 12/13/2022]
Abstract
Chronic alcohol consumption is frequently accompanied by cerebellar degeneration. The exact aetiology of alcoholic cerebellar degeneration is still a matter of debate. The aim of the present study was to investigate whether patients with chronic alcohol consumption exhibit a decrease in dentate nuclei intensity as measured by MRI, and if so, whether this decrease correlates with cerebellar atrophy as revealed by MR imaging or with clinical signs of cerebellar ataxia. A decrease in dentate nuclei intensity would indirectly indicate that iron accumulation, and therefore, oxidative stress may play a role in alcoholic cerebellar degeneration. MRI of 45 alcoholics and 44 age and sex-matched healthy control subjects was performed using a 3D-T1-weighted fast low angle shot (FLASH) echo sequence. Signal intensities of the dentate nuclei and cerebellar white matter were bilaterally measured. Planimetric measurements of cerebellar size were performed using a 3D-T1-weighted magnetization prepared rapid acquisition gradient echo (MPRAGE) sequence. Results demonstrated that dentate nuclei intensity was not significantly decreased in patients with chronic alcohol consumption (mean +/- SD signal intensity 65.36 +/- 13.0) if compared with control subjects (mean +/- SD signal intensity 68.95 +/- 9.4) (p = 0.15). Dentate nuclei intensity did not correlate with cerebellar size neither in control subjects nor in alcoholics. In contrast, vitamin B1 level correlated with cerebellar size in alcoholics even if the vitamin B1 concentration was within normal values (r = 0.344, p = 0.028). These results support the view that thiamine deficiency rather than direct neurotoxic effects of alcohol is the main causative factor for the development of alcoholic cerebellar degeneration.
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Affiliation(s)
- Matthias Maschke
- Department of Neurology, University Duisburg-Essen, Hufelandstr. 55, 45122 Essen, Germany.
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10
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Del Negro A, Dantas CD, Zanardi V, Montenegro MA, Cendes F. [Dose-dependent relationship of chronic use of phenytoin and cerebellar atrophy in patients with epilepsy]. ARQUIVOS DE NEURO-PSIQUIATRIA 2000; 58:276-81. [PMID: 10849627 DOI: 10.1590/s0004-282x2000000200012] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
Abstract
The chronic treatment with phenytoin or the acute intoxication by this drug may cause permanent cerebellar injury with atrophy of cerebellum vermis and hemispheres, which can be detected by neuroimaging studies. The aim of the present study was to investigate the correlation between the dosage and duration of treatment with phenytoin and the occurrence of cerebellar atrophy. Sixty-six patients were studied and had their tomographies analyzed for cerebellar atrophy. Of the 66 patients studied, 18 had moderate/severe atrophy, 15 had mild atrophy and 33 were considered to be normal. The patients with moderate/severe atrophy were those with higher exposure to phenytoin (longer duration of treatment and higher total dosage) showing statistically significant difference when compared to patients with mild atrophy or without atrophy (p=0. 02). Further, the patients with moderate/severe atrophy had serum levels of phenytoin statistically higher than those of patients with mild atrophy or without atrophy (p = 0.008). There was no association between other antiepileptic drugs dosage or duration of treatment and degree of cerebellar atrophy. We also found that older patients had cerebellar atrophy more frequently, indicating that age or duration of the seizure disorder may also be important in the determination of cerebellar degeneration in these patients. We conclude that although there is a possibility that repeated seizures contribute to cerebellar damage, long term exposure to phenytoin, particularly in high doses and toxic serum levels, cause cerebellar atrophy.
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Affiliation(s)
- A Del Negro
- Departamento de Neurologia e Departamento de Radiologia, Faculdade de Ciências Médicas, Universidade Estadual de Campinas
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11
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Bradford JC, Kyriakedes CG. Evaluation of the patient with seizures: an evidence based approach. Emerg Med Clin North Am 1999; 17:203-20, ix-x. [PMID: 10101347 DOI: 10.1016/s0733-8627(05)70053-5] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
Statistics tell us that as many as 1 in 20 members of the population will suffer a seizure at some point in their lifetime, a figure which becomes even more likely if one lives to the age of 80. Thus, a careful evidence based approach to the patient with seizure is immensely useful to the emergency physician. The authors evaluate current studies on the subject, discuss seizures as they relate to specific patient groups, and, ultimately, make recommendations on this important subject.
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Affiliation(s)
- J C Bradford
- Department of Emergency Medicine, Northeastern Ohio Universities College of Medicine, Rootstown, USA
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12
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Abe K, Nishikawa Y, Fujimura H, Toyooka K, Kaido M, Yorifuji S, Yanagihara T. Clinical and magnetic resonance image correlation in idiopathic cerebellar ataxia. J Neurol Sci 1995; 133:53-60. [PMID: 8583232 DOI: 10.1016/0022-510x(95)00133-m] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/31/2023]
Abstract
Sixty-one patients who fulfilled the clinical criteria for idiopathic cerebellar ataxia and who had symptoms at least for 3 years were examined clinically and by magnetic resonance imaging (MRI). Based on the clinical signs, they were divided into patients with pure cerebellar signs (Group 1), patients with additional mild rigidity and/or hyperreflexia (Group 2) and patients with additional severe rigidity and hypokinesia (Group 3). Patients in Group 1 had milder disability and better prognosis than patients in Group 2 or Group 3 (ataxic score: 14.9 vs. 28.6 and 36.0; annual progression ratio: 0.26 vs. 0.65 and 0.70, respectively). We measured the area of the cerebellar vermis, ventral pons and dorsal brainstem on midsagittal T1-weighted MR images for all patients and age- and sex-matched controls. The cerebellar vermis as well as the ventral pons of patients were significantly smaller than corresponding structures in controls (p < 0.001). The ventral pons of patients in Group 2 and Group 3 was significantly smaller than that of patients in Group 1 (p < 0.0001, respectively), and the dorsal brainstem of patients in Group 2 and Group 3 was also significantly smaller than that of patients in Group 1 (p < 0.001, respectively). The ventral pons of patients in Group 3 was significantly smaller than that of patients in Group 2 (p < 0.05) as well. There was a significant correlation between the area of the ventral pons and the annual progression ratio (p < 0.001). With MRI, slight but definite hyperintensities were demonstrated in the pontine base and the medulla of 22 patients on proton density images. In the longitudinal study, patients in Group 2 and Group 3 had atrophy of the ventral pons already at an early stage. The ventral pons of patients in Group 3 was smaller at the initial MR examination than that of patients in Group 2. These observations suggest that patients with smaller ventral pons may have rapid progression and poor prognosis. Thus, even a relatively simple quantitation of the area of the ventral pons may be useful to predict the prognosis of patients, in addition to neurologic assessment at intervals.
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Affiliation(s)
- K Abe
- Department of Neurology, Osaka University Medical School, Japan
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13
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Rodriguez ME, Artieda J, Zubieta JL, Obeso JA. Reflex myoclonus in olivopontocerebellar atrophy. J Neurol Neurosurg Psychiatry 1994; 57:316-9. [PMID: 8158179 PMCID: PMC1072821 DOI: 10.1136/jnnp.57.3.316] [Citation(s) in RCA: 42] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/29/2023]
Abstract
The presence of reflex myoclonus in response to touching and pin-pricking the wrist or stretching the fingers and to photic stimulation was assessed in 24 patients with a presumed diagnosis of olivopontocerebellar atrophy (OPCA) and in 30 age matched control subjects. Reflex myoclonus to soma-esthetic stimulation was found in 23 patients and in none of the controls. Photic myoclonus was present in 12 patients and in none of the controls. Electrophysiological study of the reflex myoclonus showed enhanced (> 10 microV) somatosensory evoked potentials and an associated reflex electromyographic discharge (C-wave) in 15 patients. These findings indicate that reflex myoclonus is common in OPCA and probably of cortical origin.
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Affiliation(s)
- M E Rodriguez
- Clinica Universitaria, University of Navarra, Pamplona, Spain
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14
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Abstract
Recent studies suggest that abnormal melatonin functions may be implicated in the pathophysiology of schizophrenia. Since there is evidence that the presence of pineal calcification (PC) may relate, among other factors, to disturbances in melatonin secretion, I investigated in 23 chronic institutionalized schizophrenic patients the relationship of PC size on CT scan to the mode of onset of schizophrenia which carries both developmental and prognostic significance. Patients with gradual onset schizophrenia had PC size that was significantly larger than those with sudden onset (8.94 +/- 3.96 mm vs. 4.80 +/- 1.75 mm p < .025). These findings suggest that the nature of onset of schizophrenia may be influenced by the activity of the pineal gland, which may exert a role in the development and prognosis of the illness.
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Affiliation(s)
- R Sandyk
- Department of Psychiatry, Albert Einstein College of Medicine, Bronx, NY 10461
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15
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Abstract
Blood thiamine levels in ataxia patients were studied. No significant differences were found between 30 patients with Friedreich's ataxia and 29 patients with olivopontocerebellar atrophy (OPCA) compared with control subjects. Both OPCA and Friedreich's ataxia patients presented significantly lower cerebrospinal fluid thiamine levels than their controls (p less than 0.001 and p less than 0.04 respectively). These results, discussed in terms of the high degree of cerebellar atrophy on CT scans in OPCA v Friedreich's ataxia patients, seem to correlate with cerebellar thiamine turnover and content.
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Affiliation(s)
- O L Pedraza
- Neurology Service, Hôtel-Dieu de Montréal, Quebec, Canada
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16
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Agartz I, Marions O, Sääf J, Wahlund LO, Wetterberg L. Visual rating of magnetic resonance images of human cerebrospinal fluid spaces and white brain matter: relation to sex and age in healthy volunteers. Magn Reson Imaging 1992; 10:135-42. [PMID: 1545673 DOI: 10.1016/0730-725x(92)90383-b] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/27/2022]
Abstract
The size of the cerebrospinal fluid spaces and the occurrence of white matter lesions were estimated from the intracranial volumes of 76 apparently healthy adult volunteers of different ages using 0.02-T/0.8-MHz magnetic resonance imaging. A relation between the occurrence of white matter lesions and the size of cerebrospinal fluid spaces independent of age could not be demonstrated. In men, white matter changes were more numerous and lateral ventricular size was larger, but sex differences were not statistically significant except for lateral ventricular size. The results confirm that age is the most significant parameter correlated with alterations in brain anatomy over time. Body mass and other clinical parameters were not influential factors in the present material.
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Affiliation(s)
- I Agartz
- Karolinska Institute, Department of Psychiatry, St. Göran's Hospital, Stockholm, Sweden
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Kay SR, Sandyk R, Merriam AE. Neuroradiological facets of cognitive abnormality in schizophrenia. Int J Neurosci 1991; 58:83-93. [PMID: 1938177 DOI: 10.3109/00207459108987185] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
Abstract
Cognitive abnormality has long been regarded as a core feature of schizophrenia, but its nature and etiopathology have been poorly understood. Predicated on new tests that characterize fundamental cognitive impairments, we investigated their relationship to four neuroradiological markers that have been previously implicated in schizophrenia: choroid plexus calcification, cerebellar atrophy, third ventricle enlargement, and pineal calcification. Twenty-three chronic schizophrenic inpatients meeting RDC and DSM-III diagnostic criteria were stabilized on chlorpromazine and assessed independently on a cognitive battery and on CT scan. The results indicated that all four neuroradiological variables were independent of one another and of demographic, historical, and general intellectual measures. The neuroradiological assessments, however, correlated significantly with different cognitive parameters, implying separate pathophysiological bases for distinct profiles of cognitive abnormality. The findings support a "dual-process model" of cognitive dysfunction that posits developmental and arousal-related components which may, more generally, underlie the positive-negative dimension of schizophrenia.
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Affiliation(s)
- S R Kay
- Department of Psychiatry, Albert Einstein College of Medicine/Montefiore Medical Center, Bronx, N.Y. 10461
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18
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Sandyk R, Kay SR, Merriam AE. Atrophy of the cerebellar vermis: relevance to the symptoms of schizophrenia. Int J Neurosci 1991; 57:205-12. [PMID: 1938163 DOI: 10.3109/00207459109150694] [Citation(s) in RCA: 29] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
Abstract
Degeneration of the cerebellar vermis is a common pathological and neuroradiological feature of chronic schizophrenia, but its relationship to symptoms of the disease are poorly understood. We investigated the relationship of vermal cerebellar atrophy on CT scan to features of positive (productive) and negative (defect) dimensions of schizophrenia as well as to symptoms of general psychopathology in a sample of 23 chronic schizophrenic patients. For comparison, we also studied the relationship of third ventricular width (TVW), which reflects periventricular and diencephalic atrophy, to these features of schizophrenic symptomatology. Vermal cerebellar atrophy was found in 43.5% of patients and correlated significantly with general psychopathology and, more specifically, with feelings of guilt and disturbance of volition. It was unrelated to global positive or negative features of schizophrenia. By contrast, TVW was uniquely associated with global negative syndrome. These observations indicate that degeneration of the cerebellar vermis and enlargement of the third ventricle are integral aspects of schizophrenia, and that each contributes independently to specific clinical aspects of the disease.
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Affiliation(s)
- R Sandyk
- Department of Psychiatry, Albert Einstein College of Medicine/Montefiore Medical Center, Bronx, New York, NY 10461
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19
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Klockgether T, Schroth G, Diener HC, Dichgans J. Idiopathic cerebellar ataxia of late onset: natural history and MRI morphology. J Neurol Neurosurg Psychiatry 1990; 53:297-305. [PMID: 2341843 PMCID: PMC1014167 DOI: 10.1136/jnnp.53.4.297] [Citation(s) in RCA: 125] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/31/2022]
Abstract
Twenty eight patients with the clinical diagnosis of idiopathic late onset cerebellar ataxia were examined clinically and by magnetic resonance imaging (MRI) or computed tomography (CT). In addition, the clinical records of all patients were analysed retrospectively. On the basis of their clinical presentation they were subdivided into patients with a pure cerebellar syndrome (n = 9) and patients with a cerebellar syndrome and additional non-cerebellar symptoms (n = 13). No attempts were made to classify patients with a disease duration of less than four years (n = 6) because the retrospective analysis showed that the disease started almost invariably with a pure cerebellar syndrome and additional symptoms came later. Patients with a lasting pure cerebellar syndrome had a significantly better prognosis than patients with additional non-cerebellar involvement (annual progression rate rate: 0.40 versus 0.80). Calculated median lifetime from onset of symptoms was 20.7 years in patients with a pure cerebellar syndrome and 7.7 years in patients with additional non-cerebellar symptoms. Among the latter, disease progression was faster the earlier non-cerebellar symptoms occurred. All of them presented with Parkinsonian symptoms, whereas bulbar symptoms, vertical gaze paresis, pyramidal deficits, dementia and urinary incontinence were encountered less frequently. MRI or CT showed cerebellar atrophy without apparent involvement of brainstem structures in all patients with a pure cerebellar syndrome suggesting the diagnosis of cerebellar cortical atrophy (CA). The majority of the patients with additional non-cerebellar symptoms had evidence of an atrophy of the cerebellum and the brainstem suggesting the presence of olivo-ponto-cerebellar atrophy (OPCA). In two of them, however, MRI morphology was not compatible with the diagnosis of OPCA.
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Affiliation(s)
- T Klockgether
- Department of Neurology, University of Tübingen, West Germany
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20
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Gallucci M, Splendiani A, Bozzao A, Baldassarre M, Zobel BB, Masciocchi C, Passariello R. MR imaging of degenerative disorders of brainstem and cerebellum. Magn Reson Imaging 1990; 8:117-22. [PMID: 2338892 DOI: 10.1016/0730-725x(90)90244-v] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/31/2022]
Abstract
In pathologies such as progressive ataxias, clinical criteria are often not enough to provide the correct diagnosis. Therefore, we studied by means of MRI 30 normal volunteers and 19 patients affected by different progressive ataxias, diagnosed by typical standards. On all the subjects measurements were performed in order to identify normal and pathological ranges. Our experience confirms that typical involvement can be detected in the different kinds of progressive ataxias. Peculiar compromissions were found in spinocerebellar diseases, able to differentiate them from the forms mostly involving the cerebellum and brainstem. The differentiation between typical Friedreich and non-Friedreich diseases can also be suggested in most cases.
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Affiliation(s)
- M Gallucci
- Department of Radiology, Università dell'Aquila, Italy
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21
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Botez MI, Botez T, Elie R, Attig E. Role of the cerebellum in complex human behavior. ITALIAN JOURNAL OF NEUROLOGICAL SCIENCES 1989; 10:291-300. [PMID: 2767936 DOI: 10.1007/bf02333774] [Citation(s) in RCA: 126] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/02/2023]
Abstract
Thirty-three outpatient epileptics with normal CT scans (group 1) and 31 patients with cerebellar and brain stem (CBS) atrophy (group 2) were randomly included in this study. There were no significant statistical differences between the groups with regard to age, education, and number of grand mal and other seizures. Statistical analyses showed that group 2 had a longer history of epilepsy with a consequently longer duration of phenytoin (PHT) consumption. Neuropsychological assessment revealed lower performance by this group on the following measures: full I.Q. scale, verbal I.Q. scale, performance I.Q. scale, information, arithmetic, block design, object assembly, digit symbol, Stroop test forms I and II, the B-M dexterity test, and the simple visual and auditory reaction time. No significant differences were observed between the two groups for the remaining 5 subtests from the WAIS scale, for the immediate recall and the delayed recall subtests belonging to Wechsler memory scale as well as for visual and auditory movement time. Analysis of the composite scores of neuropsychological performance showed that the cerebellum interferes with the following complex behavioral functions: (i) visuo-spatial organization for a concrete task, a function related to the cerebello-parietal loops' (ii) planning and programming of daily activities, a function related to the cerebello-frontal loops; and (iii) the speed of information processing, a mainly subcortical function.
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Affiliation(s)
- M I Botez
- Laboratoire de Neuropsychologie, Hôtel-Dieu de Montréal affilié à l'Université de Montréal et Centre de Recherches, Hôpital Louis-H. Lafontaine, Québec, Canada
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22
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Yaqub BA, Daif AK, Panayiotopoulos CP. Pancerebellar syndrome in heat stroke: clinical course and CT scan findings. Neuroradiology 1987; 29:294-6. [PMID: 3614627 DOI: 10.1007/bf00451771] [Citation(s) in RCA: 25] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/06/2023]
Abstract
We present the clinical course and computerized tomography (CT) findings in a case of pancerebellar syndrome due to heat stroke at the Mekkah Pilgrimage. CT scan was initially normal, but five months later, a considerable generalized cerebellar atrophy was seen despite remarkable clinical improvement.
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23
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Gutzmann H, Kühl KP. Emotion control and cerebellar atrophy in senile dementia. Arch Gerontol Geriatr 1987; 6:61-71. [PMID: 2884937 DOI: 10.1016/0167-4943(87)90039-2] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/22/1986] [Revised: 11/21/1986] [Accepted: 11/24/1986] [Indexed: 01/03/2023]
Abstract
New research increasingly indicates that the cerebellum coordinates and integrates a wide range of processes not confined to the motor sphere. Since satisfying correlations between affective disturbances and signs of cerebral atrophy have not been found, the relationship of CT-indicated cerebellar atrophy to certain psychopathologic indicators, in patients suffering from senile dementia, has been studied. The patients participating in the study were 21 women and 18 men with a mean age of 69.4 years. Each patient showed a definite pathologic score in at least one cerebral CT-measurement. The Spearman rank correlations between affective incontinency, lability of affect, and cerebellar atrophy suggest substantial relationships between affective symptoms and cerebellar pathology. This seems all the more remarkable since all of the various indicators of cerebral cortical and subcortical atrophy lacked to show any strong relationship to affective symptoms at all. The implications of the findings for research and clinical purposes are discussed.
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24
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Wolfson LI, Whipple R, Amerman P, Kleinberg A. Stressing the postural response. A quantitative method for testing balance. J Am Geriatr Soc 1986; 34:845-50. [PMID: 3782696 DOI: 10.1111/j.1532-5415.1986.tb07256.x] [Citation(s) in RCA: 136] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/07/2023]
Abstract
Using a series of graded destabilizing forces, we have developed a simple quantitative test of the postural response: the Postural Stress Test (PST). Suitable for widespread testing of elderly subjects, the test evaluates the subject's ability to avoid a fall as well as the appropriateness of the response. We have determined that by comparison with young subjects, the elderly controls have compromised although functionally effective balance. On the other hand, the balance response was severely compromised in half of the individuals with a history of falls. Experience with the Postural Stress Test suggests it will predict those elderly individuals with a tendency to fall as well as provide a simple mechanism for studying the balance response in the elderly.
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25
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Rindi G, Imarisio L, Patrini C. Effects of acute and chronic ethanol administration on regional thiamin pyrophosphokinase activity of the rat brain. Biochem Pharmacol 1986; 35:3903-8. [PMID: 3022743 DOI: 10.1016/0006-2952(86)90002-x] [Citation(s) in RCA: 29] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/03/2023]
Abstract
Thiamin pyrophosphokinase (TPKase) activity was determined in supernatants of cerebral cortex, cerebellum, pons, medulla, hypothalamus and corpus callosum homogenates obtained from normal rats and from rats given ethanol acutely (a single dose of 4.7 g X kg-1 body wt) or chronically (4.7 g X kg-1 body wt daily for 35 days) by gastric gavage. Regional cell densities (derived from DNA content) and protein contents were also determined. TPKase was detected in all brain regions investigated, the highest activity being found in the cerebellum or in the pons depending on whether it was expressed per mg of protein or per number of cells, respectively. In samples taken following acute ethanol administration protein content was unaffected, while TPKase activity was significantly reduced in the cerebellum, cerebral cortex and hypothalamus at 90 min and in the cerebellum and cerebral cortex at 300 min. Chronic ethanol intake was associated with a significant decrease in regional cell densities, protein contents and TPKase activity. The addition of ethanol to the incubation medium of normal tissue supernatant caused a dose-dependent inhibition of TPKase activity. These results suggest that ethanol markedly impairs thiamin cellular utilization, which may result in depression of brain metabolism.
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26
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Mousa AR, Al-Din AS, Al-Nassar KE, Al-Rifai KM, Rudwan M, Sunba MS, Behbehani K. Autosomally inherited recessive spastic ataxia, macular corneal dystrophy, congenital cataracts, myopia and vertically oval temporally tilted discs. Report of a Bedouin family--a new syndrome. J Neurol Sci 1986; 76:105-21. [PMID: 3465874 DOI: 10.1016/0022-510x(86)90145-0] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/05/2023]
Abstract
A Bedouin family is described with an unusual form of spinocerebellar degeneration. Spastic ataxia was found to be associated with congenital cataracts, macular corneal dystrophy and non-axial myopia, in the absence of retardation of somatic or mental maturation. Immunological abnormalities were common. Genetic analysis revealed that the pedigree is expressing the transmission and segregation of a single mutant autosomal recessive gene.
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27
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Marshak TL, Avrushchenko MS. Changes in size of Purkinje cell nucleoli in the dog cerebellum in the postresuscitation period. Bull Exp Biol Med 1986. [DOI: 10.1007/bf00851072] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/26/2022]
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Scholz E, Diener HC, Dichgans J, Langohr HD, Schied W, Schupmann A. Incidence of peripheral neuropathy and cerebellar ataxia in chronic alcoholics. J Neurol 1986; 233:212-7. [PMID: 3018178 DOI: 10.1007/bf00314021] [Citation(s) in RCA: 43] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/03/2023]
Abstract
A total of 78 chronic alcoholics were examined neurologically as well as by electroneurography, myography and posturography. Clinical signs of peripheral neuropathy were detected in 45% of these patients, with electromyographic and neurographic abnormality in 67% and 55% respectively. Clinical signs of cerebellar ataxia were found in 33% of our patients, whereas posturographic measurements of increased sway were recorded in 69%. The posturographic characteristics of cerebellar anterior lobe atrophy were observed in two-thirds of the latter patients. The severity of cerebellar-ataxia did not correlate with the degree of neuropathy. This lack of correlation is interpreted as an indication of different pathogenetic mechanisms acting on peripheral nerves and cerebellum.
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Abstract
Forty-five alcoholics were examined clinically and by CT scanning in order to evaluate the relationship between ataxia, cerebellar atrophy and peripheral neuropathy. A significant relationship was found between ataxia and cerebellar atrophy as well as between ataxia and peripheral neuropathy.
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Diener HC, Müller A, Thron A, Poremba M, Dichgans J, Rapp H. Correlation of clinical signs with CT findings in patients with cerebellar disease. J Neurol 1986; 233:5-12. [PMID: 3950666 DOI: 10.1007/bf00313982] [Citation(s) in RCA: 29] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/08/2023]
Abstract
The severity of cerebellar signs and the degree of cerebellar atrophy depicted by computed tomography (CT) were independently graded in 108 patients with cerebellar disorders. The overall agreement between these independently scaled measures was only 28%. In patients with involvement of the cerebellar hemispheres and anterior lobe, clinical signs tended to be more pronounced than the cerebellar atrophy revealed by CT. The opposite was true for patients with lesions of the caudal vermis. Patients with Friedreich's ataxia had no or only minor CT abnormalities. Close correlation between the degree of infra- and supratentorial atrophy was found only in chronic alcoholics. The poor correlation between changes in cerebellar structure detected by CT and clinical disability suggests the need for caution in CT interpretation.
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31
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Andreula CF, Camicia M, Lorusso A, D'Aprile P, Federico F, Brindicci D, Carella A. Clinical and CT parameters in degenerative cerebellar atrophy in aged patients. Neuroradiology 1984; 26:29-30. [PMID: 6738839 DOI: 10.1007/bf00328199] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/21/2023]
Abstract
Fifteen cases of degenerative cerebellar atrophy are presented together with the criteria used to assess the diagnosis of cerebellar atrophy by computed tomography.
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32
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Dewan MJ, Pandurangi AK, Lee SH, Ramachandran T, Levy BF, Boucher M, Yozawitz A, Major L. Cerebellar morphology in chronic schizophrenic patients: a controlled computed tomography study. Psychiatry Res 1983; 10:97-103. [PMID: 6581493 DOI: 10.1016/0165-1781(83)90108-7] [Citation(s) in RCA: 31] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/20/2023]
Abstract
Simple, objective, linear, and density measures were used to evaluate by computed tomography (CT) the cerebellum-fourth ventricular region of 23 chronic schizophrenic patients and 23 normal controls. Our data suggest that a subgroup of chronic schizophrenic patients have cerebellar atrophy associated with a strong but nonsignificant trend toward increased cerebellar density. The implications of these findings are discussed in view of previous CT and neuropathological studies.
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Abstract
Eight parameters of cranial computed tomograms were examined from 121 controls, 33 patients suffering from paleocerebellar atrophy and 53 with widespread cerebellar atrophy. With the aid of step-by-step linear discriminant analysis the most important parameters were found and their normal and limiting values were calculated. With the help of correlation analysis the correlation between normal values and the age of the controls was investigated.
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34
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Loizou LA, Rolfe EB, Hewazy H. Cranial computed tomography in the diagnosis of multiple sclerosis. J Neurol Neurosurg Psychiatry 1982; 45:905-12. [PMID: 6292371 PMCID: PMC491595 DOI: 10.1136/jnnp.45.10.905] [Citation(s) in RCA: 27] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/19/2023]
Abstract
A group of 202 patients with suspected, probable or definite multiple sclerosis was studied, using cranial computed tomography (CT). Atrophy alone, or in combination with white-matter and periventricular lucencies, and areas of contrast enhancement, were the main abnormal findings in 52% of patients. Atrophy was detected in 44% of patients, and its frequency and severity correlated with disease duration up to 10 years, age, and disease category. Atrophic changes in the brainstem and cerebellum could be correlated with clinical data more often than supratentorial atrophy could be correlated with features such as dementia or mood changes. Lucencies in the white matter, thought to represent areas of demyelination, were noted in 21% of patients, and only a proportion of these lesions could be correlated with clinical data, the others being clinically silent. Contrast enhancement was seen in a small proportion of white-matter lesions, and was independent of disease activity and steroid medication. Electrophysiological tests and cerebrospinal fluid analysis showed a higher yield of abnormality than CT scanning in cases with suspected or possible multiple sclerosis, though in such patients CT scanning excluded alternative cerebral atrophy. Modifications of the technique of CT scanning may improve the detection rate of white-matter lesions, thereby enhancing the value of CT as a diagnostic tool in the study of patients with multiple sclerosis.
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Berciano J, Amado JA, Freijanes J, Rebollo M, Vaquero A. Familial cerebellar ataxia and hypogonadotropic hypogonadism: evidence for hypothalamic LHRH deficiency. J Neurol Neurosurg Psychiatry 1982; 45:747-51. [PMID: 6813427 PMCID: PMC1083171 DOI: 10.1136/jnnp.45.8.747] [Citation(s) in RCA: 43] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/22/2023]
Abstract
A family with familial cerebellar ataxia and hypogonadotropic hypogonadism is described. The condition was inherited as an autosomal recessive defect. CT scan in one case revealed cerebellar and brain stem atrophy. Endocrinological tests showed abnormalities only in two patients who were clinically affected. In both cases raised gonadotropic levels were found after repetitive stimulation with luteining hormone-releasing hormone which suggests that the hypogonadism was due to a primary hypothalamic disturbance.
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