Degree of cerebellar ataxia correlates with three-dimensional mri-based cerebellar volume in pure cerebellar degeneration

The aim of the present study was to compare the severity of cerebellar ataxia as measured by the International Cooperative Ataxia Rating Scale (ICARS) by Trouillas et al. [ J Neurol Sci 1997;145:205-211] with the cerebellar volume in chronic cerebellar disease. Fifteen patients with pure cerebellar degeneration were investigated. Seven patients suffered from spinocerebellar ataxia type 6, 5 from idiopathic late-onset cerebellar ataxia, 2 from autosomal dominant cerebellar ataxia type III and 1 from episodic ataxia type 2. Volumetric analysis was based on individual three-dimensional MR images. Total ICARS score significantly inversely correlated with the cerebellar volume (r = -0.805, p < 0.0001), correlations between ICARS subscores and cerebellar volume were significant for upper and lower limb ataxia, ataxia of posture and gait, and dysarthria, but not for the oculomotor subscore. The results suggest that the degree of cerebellar atrophy in pure cerebellar degenerative disorders is accompanied by comparable functional impairment (i.e. degree of cerebellar ataxia).     

An adult case of cerebellar mutism after removal of cerebellar hematoma]

Cerebellar mutism is a well documented complication of posterior fossa tumor surgery in children. But only one case presenting with cerebellar mutism after removal of a cerebellar hematoma in adults was reported previously. We present 64-year-old male developing cerebellar mustism after surgery for hypertensive cerebellar hemorrhage. A cerebellar hematoma was located in the right side dentate nucleus and cerebellar peduncle dominantly not extending to the cerebellar vermis. During the period of cerebellar mutism, single photon emission computed tomography revealed a decrease in the contralateral temporo-parietal cerebral blood flow. Cerebellar mutism in this case may be associated with the low blood flow in the contralateral supplementary motor cortex owing to injury to the dentate-thalamo-cortical pathway at the dentate nucleus and cerebellar peduncle. The relevant literature is reviewed.     

Unilateral cerebellar hypoplasia.

In a 48-year-old man, marked unilateral cerebellar hypoplasia was diagnosed by CT scans and angiographic examinations, later confirmed on autopsy. Clinical findings in cerebellar hypoplasia/aplasia are discussed here, along with the pathogenesis of cerebellar hypoplasia. In this case, several grand mal seizures and persistent headache were the only neurological symptoms. Repeated neurologic explorations did not reveal any abnormality. The clear demonstration of cerebellar hypoplasia associated with ipsilateral hypoplasia of the vertebral artery favors the concept of a vascular genesis of cerebellar hypoplasia/aplasia.     

小脑发育不良性节细胞瘤

研究背景小脑发育不良性节细胞瘤是小脑良性肿瘤,临床表现不典型,相对罕见。本文报告1例小脑发育不良性节细胞瘤患者,通过复习相关文献,探讨其临床病理学特征,以期提高诊断与鉴别诊断能力。方法与结果女性患者,34岁,头部MRI显示右侧小脑半球类圆形占位性病变。手术全切除病变,术中可见病变表面脑回增宽,呈黄白色,似脑组织样,质地略软,血供丰富,无包膜,与周围脑组织无粘连。组织学形态,颗粒细胞层和浦肯野细胞层被平行排列的异常有髓纤维和结构紊乱的异常神经元取代,伴血管畸形。免疫组织化学染色,异常神经元胞质表达神经元核抗原、突触素、S-100蛋白和神经元特异性烯醇化酶,胶质纤维背景表达胶质纤维酸性蛋白,神经纤维表达神经微丝蛋白,Ki-67抗原标记指数1%。最终病理诊断为小脑发育不良性节细胞瘤。术后仍呈深昏迷,共住院24 d,出院后失访。结论小脑发育不良性节细胞瘤临床相对罕见,组织学形态呈良性,影像学表现具有一定特异性,早期诊断并手术治疗十分必要,应注意与节细胞瘤、节细胞胶质瘤和低级别星形细胞瘤相鉴别。小脑发育不良性节细胞瘤患者及其家属应进行系统检查,关注有无其他器官或系统疾病或肿瘤。     

Decreased cerebellar blood flow in postinfectious acute cerebellar ataxia

OBJECTIVE: The aim of the present study was to evaluate the regional cerebral blood flow (rCBF) in patients with postinfectious acute cerebellar ataxia using single photon emission computed tomography (SPECT). METHODS: Five children with postinfectious acute cerebellar ataxia and five control subjects were examined. The distribution of rCBF was measured by SPECT imaging after intravenous administration of 123I-IMP (111 MBq). The rCBF ratio-defined as the ratio of rCBF in the region of interest (ROI) to that in the occipital cortex-was calculated for each cortical and subcortical ROI. The mean rCBF ratio of each region was then compared between the ataxic and control subjects. These patients and all control subjects were also evaluated using MRI. RESULTS: The rCBF ratio was significantly lower in the cerebellum of the ataxic patients than in the cerebellum of the control subjects (p<0.05). No abnormal cerebellar morphology and no abnormal signal intensities were found on MRI. CONCLUSION: 123I-IMP SPECT clearly demonstrated the decreased rCBF in the cerebellum of all patients with postinfectious acute cerebellar ataxia.     

Autosomal recessive cerebellar hypoplasia

Cerebellar hypoplasia is found in association with a variety of neurologic and systemic disorders. It is the primary finding in the uncommonly reported condition of autosomal recessive cerebellar hypoplasia. We describe two siblings with cerebellar hypoplasia documented in both by magnetic resonance imaging (MRI) and review the clinical features of previously reported cases of autosomal recessive cerebellar hypoplasia. The most common findings in this disorder are nonprogressive ataxia, strabismus, mental retardation, and speech delay with dysarthria. Previously reported cases have been confirmed by autopsy, pneumoencephalography, or computed tomographic (CT) scans. MRI clearly documents diffuse cerebellar hypoplasia and aids in distinguishing autosomal recessive cerebellar hypoplasia from other disorders. The pathophysiology of this disorder is uncertain, however, studies of the weaver mutant mouse (an animal model of autosomal recessive cerebellar hypoplasia) suggest that an abnormality of the Bergmann glia may lead to the observed granule cell layer deficiency in these patients. This diagnosis should be considered for children with nonprogressive ataxia and families should be made aware of the 25% recurrence risk.     

Immunofluorescent labeling of nonhuman cerebellar tissue with sera from patients with systemic cancer and paraneoplastic cerebellar degeneration

Summary Sera from patients with paraneoplastic cerebellar degeneration have been shown to contain high titers of antibody to human Purkinje cells. It is not known, however, whether these sera react with cerebellar material from species other than man. The present study was conducted to determine whether cerebellar tissue of nonhuman species could be used to screen human sera for anticerebellar antibodies and whether similarities in cerebellar antigens between nonhuman and human material might permit attempts to transmit cerebellar degeneration to experimental animals by passive transfer of patient sera. Sections of human, monkey, pig, sheep, cat, rabbit, and rat cerebellar tissue were overlaid with serial dilutions of positive sera from patients with cancer and with paraneoplastic cerebellar degeneration and were stained using indirect immunofluorescence methods. All sera stained specific Purkinje cells when reacted with monkey, pig, and rabbit cerebellar sections, but not all sera stained sheep, cat, or rat tissue. Immunofluorescent labeling of animal cerebellar tissue was less bright than that obtained with human cerebellar sections, and anticerebellar antibody titers were invariably lower when assayed with nonhuman than with human material. Although human anticerebellar antibodies react with cerebellar tissue from other animal species, patient-to-patient variation in staining is sufficiently great that not all patient sera might be suitable for passive transfer experiments, and that attempts to identify anticerebellar antibodies by reacting patient sera with nonhuman cerebellar tissue could be negative where these antibodies are in fact present and could be demonstrated using human material.     

Neuropsychological disturbances in cerebellar infarcts

BACKGROUND: Recent clinical and research reports suggest that the cerebellum may contribute to the modulation of higher cognitive functions. MATERIAL AND METHODS: We included 15 consecutive patients with isolated cerebellar infarcts (4 superior cerebellar artery territory infarcts, 1 anterior inferior cerebellar artery territory infarct and 10 posterior inferior cerebellar artery territory infarcts), all confirmed by magnetic resonance imaging. These subjects plus 15 controls matched for age, sex, and educational level underwent a neuropsychological test battery including vocabulary, similarities and block design subtests of the Wechsler Adult Intelligence Scale Revised (WAIS-R); verbal fluency tests (categorial, phonemic, and alternate categorial fluency tests); Rey's 15 auditory word learning list; the paced auditory serial addition task; the Stroop test and the Zerssen Rating Scale. One year after the cerebellar infarct, each subject with cerebellar infarct was asked to undergo a second neuropsychological examination with the same test battery. RESULTS: Patients with cerebellar infarcts exhibited significantly lower neuropsychological performances compared to those of the control group without any obvious difference between the different vascular cerebellar territories. After 1 year of follow-up, this neuropsychological impairment tended to improve. CONCLUSIONS: The pattern of deficits observed in isolated cerebellar infarcts highlights the nonmotor functions of the cerebellum and functional relationship between the cerebral cortex and the cerebellum.     

Hemispatial neglect in cerebellar stroke

Cognitive deficits can be associated with cerebellar injury. The purpose of this study is to learn 1) if unilateral cerebellar injury might also cause hemispatial neglect, and if so, 2) if there is a left versus right asymmetry, 3) if the neglect is contralesional (CN) or ipsilesional (IN), and 4) if cerebellar injury might induce neglect by disruption of cerebellar-cortical networks. Participants were 28 patients with unilateral cerebellar stroke who were assessed for neglect within 2 months after the onset of stroke. To investigate if the cerebellar-cerebral network dysfunction induced neglect, 12 patients received perfusion single photon emission computed tomography (SPECT). Eight of the participants demonstrated neglect (28.6%), four with left cerebellar strokes (three with CN and one with IN) and four with right cerebellar strokes (three with IN and one with CN). Among five patients with neglect who had undergone SPECT, only one with ipsilesional neglect showed crossed cerebello-cerebral diaschisis. Neglect induced by cerebellar stroke might be more common than previously reported. Based on the cerebellar-cerebral network hypothesis we expected neglect to be more common with left than right cerebellar injury, but there was an equal number of patients with neglect from right and left sided strokes and the SPECT scan did not provide support of this hypothesis. Thus, this hypothesis cannot also explain the equal number of subject with ipsi- and contralesional neglect and in future studies alternative hypotheses such as vestibular hypothesis will have to be explored.     

Management of acute cerebellar stroke

Acute cerebellar infarction or hemorrhage may initially manifest in a clinically indolent manner only to later deteriorate into a life-threatening neurologic catastrophe. At the other end of the spectrum, some patients with cerebellar stroke may present in a moribund comatose state. In both patient groups, it is often unclear at what point surgical intervention should be considered either to prevent further neurologic deterioration or to try to salvage a meaningful neurologic recovery. In this review, we present clinical cases that illustrate decision points in the management of patients with acute cerebellar stroke, with emphasis on clinical and imaging characteristics. We conclude with an analysis of clinical decision making in the management of patients with space-occupying cerebellar stroke. The management of acute cerebellar infarction or hemorrhage often requires difficult and prompt decisions by treating neurologists, and certain easily identifiable clinical and imaging findings may assist in appropriate patient triage and timely neurosurgical intervention.     

The "dark cerebellar sign"

We present and discuss the "dark cerebellar sign" in contrast to the well known "white cerebellar sign". The "white cerebellar sign" relates to a normal cerebellum which appears hyperdense in contrast to a pathological hypodense cerebrum on computer tomography (CT). We present a child with a "dark cerebellar sign" characterized by an ischemic or edematous cerebellum which appears hypodense in contrast to a normal relatively dense cerebrum. Isolated infarction of the cerebellum is a rare finding in premature neonates. Even rarer, cerebellar infarction may be observed in children and young adults due to an overdose of tricyclic antidepressants (TCA). The reason for the selective cerebellar infarction with TCA intoxication is still unknown. Our case shows that TCA intoxication should be included in the differential diagnosis of children with a "dark cerebellar sign".     

Myoclonic cerebellar dyssynergia (Ramsay-Hunt syndrome) and cerebellar telangiectasia

A 8 year-old girl presented with generalized epileptic seizures followed by the progressive onset of myoclonic jerks, sometimes associated with willed movements, and a static and kinetic cerebellar syndrome without conspicuous intellectual impairment. Death occurred 10 years after the onset of the disorders. There was no family history. Neuropathological studies showed lesions confined to the cerebellum. Diffuse and bilateral telangiectases were present in the cerebellar white matter. They were associated with patchy cortical alterations of the distal parts of some folia involving mainly the granule-cells ans sparing the Purkinje cells. No Lafora bodies and no abnormal lipofuscin storage were observed. The dentate nuclei, superior cerebellar peduncles and red nuclei were normal as were the inferior olives and inferior cerebellar peduncles. The spino-cerebellar tracts were unaffected. This case confirms the hypothesis that dyssynergia cerebellaris myoclonica corresponds only to a clinical entity. It may be encountered in various degenerative or metabolic disorders involving the cerebellum and/or its pathways. To our knowledge the association of a Ramsay Hunt syndrome with a vascular malformation has not been previously reported.     

Psychiatric symptoms and cerebellar pathology

The authors describe three patients hospitalized for psychiatric disorders, all of whom had cerebellar lesions. Referring to recent research on nonmotor cerebellar functions, the authors suggest that patients with cerebellar lesions may develop an organic brain syndrome that closely resembles the organic brain syndrome associated with cerebral cortical lesions.     

Clinical spectrum associated with cerebellar hypoplasia

We reviewed 45 children with cerebellar hypoplasia on magnetic resonance imaging to identify clinical features associated with cerebellar hypoplasia. We then studied children presenting with any likely associated clinical feature of cerebellar hypoplasia previously observed or reported. Two hundred fifty-one children, with one or more of these features, exhibited no cerebellar hypoplasia on imaging. We compared the children with cerebellar hypoplasia with those without cerebellar hypoplasia. Logistic regression and Pearson’s χ² test were used.

Of the 45 children with cerebellar hypoplasia, 39 exhibited developmental delay; 24, speech delay; 25, seizures; nine, microcephaly; 22, hypotonia; 22, ataxia and impaired coordination; four, abnormal movements (tremor or titubation); 13, hypertonia; eight, autistic features; and 18, ocular signs (nystagmus, strabismus, and abnormal ocular movements). Statistically significant clinical features of children with cerebellar hypoplasia compared with those without were development and speech delay, microcephaly, abnormal movements, ataxia and impaired coordination, autistic features, hypotonia, and ocular signs. The regression combination of speech delay, ataxia, hypotonia, autistic features, and ocular signs correctly predicted 86% of those with cerebellar hypoplasia.

Main clinical features of cerebellar hypoplasia are developmental or speech delay, autistic features, ataxia, hypotonia, and ocular signs.     

Dexterity in cerebellar agenesis

Given the well established role of the cerebellum in motor control, deficient motor performance during life time appears to be common in cerebellar agenesis. However, behavioural data on motor performance in living subjects with cerebellar agenesis are scarce. Dexterity during object manipulation was comparatively investigated in a 63-year old female with cerebellar agenesis and three healthy age- and gender-matched control subjects. Participants performed a transport task with an instrumented object and caught a weight that was dropped into a hand-held receptacle either expectedly from the opposite hand or unexpectedly from the experimenter's hand. Compared to healthy subjects, the subject with cerebellar agenesis generated greater grasping forces. For the transport task the patient showed a clear impairment of the predictive adjustment of grasping forces to the differential loading requirements of movement direction. For the weight-catching task, the patient established an accurate reactive mode of control when the weight was dropped unexpectedly. In case the weight was dropped expectedly from the opposite hand, predictive control mechanisms were severely disturbed in the subject with cerebellar agenesis. These data highlight the role of the cerebellum for predictive force control and are interpreted within the concept of internal models reflecting the causal relationship between actions and their consequences.     

Cortical cerebellar degeneration with testicular neoplasm

Summary Cortical cerebellar degeneration was found in a 28-year-old man with testicular neoplasm. The patient, who had undergone a left orchidectomy for the testicular tumor, developed progressive cerebellar symptoms with mental changes 7 months later. The autopsy revealed the spread of a malignant germ cell tumor of the testis, and cortical cerebellar degeneration in the central nervous system (CNS) which was characterized by almost complete loss of Purkinje cells and degeneration of the both dentate nuclei and superior cerebellar peduncles.The present case is the first of cortical cerebellar degeneration combined with testicular neoplasms.     

Benign cerebellar hemorrhages

Two patients with cerebellar hemorrhage and a benign outcome stress the potential for spontaneous resolution and clinical recovery in some cases of this disorder. In each patient, resolution of the hemorrhage was noted on the computed tomographic scan. Computed tomography may detect a cerebellar hemorrhage even though on clinical grounds a brainstem localization is favored.     

Acute cerebellar ataxia in children

Acute cerebellar ataxia is a benign syndrome usually occurring after an acute febrile disease. In a few cases neuroradiological investigations reveal cerebellar alterations. Clinical and neuroradiological involvement of the brain stem has rarely been reported in the literature. We present five cases of acute cerebellar ataxia. In two cases the cerebellar symptomatology was associated with neurological signs of brain stem involvement. CT scans did not show any pathologic findings in three patients. MRI disclosed cerebellar or brain stem alterations in all the patients. Clinical and neuroradiological findings allow differentiation of this pathologic entity from other demyelinating or dysmyelinating diseases. The value of MRI in detection and localization of the lesions and in following their evolution is emphasized. Received: 4 December 1996 Revised: 20 May 1997     

An autopsied case of idiopathic late cortical cerebellar atrophy--comparison with other cortical cerebellar atrophy

A 68-year-old man without familial history developed ataxic gait and sensory disturbance in the lower extremities. At the age of 74, neurological examination revealed marked cerebellar ataxia of all limbs, dysarthria, sensory disturbance of glove and stocking type in the extremities, and slight neurogenic muscular atrophy. There were no mental deterioration and dysautonomia. He died of pneumonia at the age of 74. Neuropathological findings. The cerebellum was decreased in size. Microscopically, there were severe disappearance of Purkinje cells in the cerebellar vermis and hemispheres. The molecular layer, granular cell layer, and cerebellar white matter were preserved. Neurons of the inferior olivary nuclei were also spared. In the spinal cord, there was myelin pallor in the posterior column predominant in Goll's fascicule and moderate atrophy of neurons in the anterior horn. Degeneration of the posterior roots was greater than that of the anterior roots. No abnormal findings were found in the extrapyramidal system, cranial nerves, and cerebrum. We compared this case clinicopathologically with other diseases with cortical cerebellar atrophy; alcoholic cerebellar degeneration, phenytoin intoxication, neuroleptic malignant syndrome, and subacute paraneoplastic cerebellar degeneration. In conclusion, idiopathic late cerebellar cortical atrophy (LCCA) was different clinicopathologically from the other diseases. Especially, LCCA showed the characteristic topography of Purkinje cells loss sparing the molecular and granule cell layers.