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PubMed2006

Sporadic Creutzfeldt-Jakob disease with cerebellar ataxia at onset in the UK.

OBJECTIVE: To determine the frequency, in the UK, of sporadic Creutzfeldt-Jakob Disease (sCJD) with a cerebellar ataxic onset, and to describe the clinical features of the syndrome. METHODS: A retrospective review of autopsy-proved cases of sCJD cases in the UK, 1990-2005, identifying those presenting with cerebellar features without early cognitive decline. RESULTS: 29 of 618 (5%) patients with sCJD had an isolated cerebellar onset. Mean illness duration was 9 months. Subsequently, 21 (72%) developed myoclonus and 23 (79%) developed pyramidal features. Magnetic resonance imaging showed high signal in the basal ganglia in 11 of 14 (79%) patients. 7 of 15 (47%) patients were valine homozygotic at prion protein gene (PRNP)-129. Only 8 (28%) cases were referred to the surveillance unit after death. CONCLUSION: A better definition of sCJD presenting with an isolated cerebellar syndrome might improve future case recognition and contribute to the determination of its cause.

Age of Onset↗
PubMed1989

Chronic neurological toxicity associated with exposure to volatile substances.

1. The main neurological disorders associated with chronic VSA are peripheral neuropathy, cerebellar disease, chronic encephalopathy and dementia. Apart from peripheral neuropathy, the clinical features are non-specific, evidence for solvent-related toxicity is in most cases circumstantial and there is no clear dose/response relationship. 2. Peripheral neuropathy is mainly associated with n-hexane and methyl n-butyl ketone. 3. Cerebellar disease is usually associated with toluene exposure; in the more severe cases there is often radiological evidence of irreversible cerebellar atrophy. 4. Chronic encephalopathy and dementia are the most serious consequence of solvent exposure, particularly to toluene in abusers and to mixed solvents in industrial workers. Postmortem studies in some abusers have shown generalized axonal degeneration, demyelination and brain atrophy. 5. Further studies on low level solvent exposure are needed as little is known about the neurological consequences of mild VSA, especially as regards individual susceptibility and possible interactions between solvents and other toxins such as ethanol.

Brain Diseases↗
PubMed2000

Ocular microtremor (OMT): a new neurophysiological approach to multiple sclerosis.

Using a piezoelectric transducer, the frequency and pattern of ocular microtremor (OMT) between 50 normal subjects and 50 patients with multiple sclerosis were compared. Controls were age matched. All records were analysed blindly. The frequency of OMT in the normal group was 86 (SD 6) Hz, which was significantly different from that of the multiple sclerosis group (71 (SD) 10 Hz, p<0.001). Those in the multiple sclerosis group with clinical evidence of brain stem or cerebellar disease (n=36) had an average OMT frequency of 67 (SD 9) Hz (p<0.001) compared with normal (n=86), whereas those with no evidence of brain stem or cerebellar involvement (n=14) had a frequency of 81.2 (SD 6) Hz (p<0.05, n=64). The differences between the two multiple sclerosis groups were also significant (p<0. 001, n=50). At least one abnormality (frequency and pattern) of OMT activity was seen in 78% of patients with multiple sclerosis. In the presence of brain stem or cerebellar disease 89% had abnormal records whereas in the absence of such disease 50% had abnormal records. This is the first report of the application of this technique to patients with multiple sclerosis. The results suggest that OMT activity may be of value in the assessment of multiple sclerosis.

Adult↗
PubMed1995

Myoclonic-like finger microdisplacements in patients with cerebellar deficits.

BACKGROUND: Here we assess the ability of patients with cerebellar disease to execute a simple visually-guided movement task involving tracking of a target with the index finger. METHODS: Spontaneous microdisplacements in index finger position are compared in patients with cerebellar deficits (ischemia [n = 3], multiple sclerosis [n = 3], degenerative cerebellar disease [n = 3]) and age-matched healthy subjects. Subjects were required to maintain a constant finger position relative to a stationary baseline displayed on an oscilloscope. RESULTS: Unusual transient abrupt movements (saccadic or myoclonic-like) directed with or against gravity were seen in patients whose neurological deficits were the most severe (7/9 patients). These abrupt myoclonic-like movements occurred independently of visual input, were not associated with clinically observable myoclonus, and were not detected previously in patients with Parkinson's disease. These abrupt myoclonic-like movements were not associated with abnormalities in either physiological tremor, or oscillations in finger microdisplacements induced by insertion of a delay (300-1400 ms) into the visual feedback of this finger "holding" experiment. An unexpected finding is that the results obtained for patients with cerebellar deficits by insertion of an experimental delay are not significantly different from those obtained with their age-matched controls. CONCLUSIONS: These observations suggest that abrupt myoclonic-like movements are a characteristic abnormality of patients with a variety of cerebellar deficits and emphasize the value of this simple motor tracking task for characterizing movement disorders.

Adolescent↗
PubMed2002

Evaluation of postural tremor of finger for neuromuscular diseases and its application to the classification.

The purpose of this study is to verify the features of the power spectrum of postural tremors for neuromuscular disease patients and to classify the postural tremors. The subjects were 88 neuromuscular disease patients (30 Parkinson disease (PD), 25 cerebellar disease (CER), 7 multiple sclerosis (MS), 7 neuropathy (NEU), 10 motor neuron disease (MND), 9 myopathy (MYO)). The control subjects were 12 normal young persons and 10 normal aged persons. Postural tremor was detected by accelerator sensor. Postural tremor was recorded under the two postural conditions: The subjects maintained the index finger without or with a weight load of 50 g in a horizontal position while looking at a visual target in front of the tip of the index finger. The power spectrum was calculated by an auto-regressive model (AR model). The peak frequency and the peak power were evaluated under the two conditions. Two frequency components of 8-12 Hz and 20-25 Hz appeared in the postural tremor of both normal subjects and neuromuscular disease patients. The difference of the postural tremor between the subjects mainly appeared in the 8-12 Hz component during the postural tremor with a weight load. MYO patients belonged to one group (called as group P1) due to lower peak power, CER patients belonged to one group (called as group P2) due to higher peak power, and PD and MS patients belonged to one group (called as group P3) due to lower peak frequency and higher peak power. NER and MND patients belonged to one group (called as group N which meant normal group). These results suggested that the peak frequency and the peak power of the 8-12 Hz component were changed by the conditions of both spinal reflex system and central nervous system. An oscillator within the central nervous system produced the underlying frequency of 8-12 Hz component, while the amplitude of 8-12 Hz component was governed by both spinal reflex system and central nervous system. In conclusion, the classification of postural tremor for neuromuscular disease patients was a useful index to elucidate the mechanism of tremor oscillation and to assist in clinical diagnosis of neuromuscular disease.

Adult↗
PubMed2005

An overview of the patient with ataxia.

Ataxia, a neurological sign characterized by the incoordination of voluntary movements, is the most prominent manifestation of cerebellar disease. The cardinal features of cerebellar dysfunction involve disturbances of stance, gait, eye movements, muscle tone, skilled movements, and speech. Classification and differential diagnosis of ataxic syndromes have intrinsic complexity owing to the variability in phenotypic presentations and in etiologies, which include trauma, toxic and metabolic causes, neoplasms, immune mechanisms, and genetic diseases. Pure cerebellar symptoms are rarely observed, while the clinical picture of both genetic and sporadic ataxia syndromes is sometimes complicated by the presence of extracerebellar neurological or multisystem extraneural pathology. Clinical presentation and assessment of the patients together with classification, genetic aspects, and principles in differential diagnosis of ataxias are briefly reviewed.

Ataxia↗
PubMed1979

[True and false cerebellar oculomotor manifestations (author's transl)].

The oculomotor perturbations of cerebellar affections are numerous. Their localizing value is however extremely variable: It is amongst the abnormalities of refixation that one can find the most typical cerebellar feature: such as dysmetria, macrosquare wave jerks, macrosaccadic oscillations only be seen in cerebellar disease. The ocular static of cerebellar patients is often abnormal, and affected by square wave jerks and pendular nystagmus. However, the abnormalities of cinetics are not specific.

Ataxia↗
PubMed1993

Feedback and delays in neurological diseases: a modeling study using dynamical systems.

Numerous regulatory mechanisms in motor control involve the presence of time delays in the controlled behavior of the system. Experimentally, we have shown that an increase of the time delay in visual feedback induces different oscillations in control subjects and in patients with neurological diseases during the performance of a simple compensatory tracking task. A preliminary model is proposed to describe the oscillations observed in control subjects and in patients with neurological diseases. The influence of delays in two feedback loops are the main components of the motor control circuitry involved in this task and are studied from an analytical and physiological perspective. We analytically determine the influence in the model of each of these delays on the stability of the finger position. In addition, the influence of stochastic elements ("noise") in the modeling equation is seen to contribute qualitatively to a more accurate reproduction of experimental traces in patients with Parkinson's disease but not in patients with cerebellar disease.

Cerebellar Diseases↗
PubMed1995

[Vogt-Koyanagi-Harada disease with cerebellar lesions demonstrated on MRI: a case report].

We reported unique magnetic resonance imaging (MRI) findings of a 57-year-old Japanese man who was diagnosed as Vogt-Koyanagi-Harada disease. This patient presented with complaints of a transient severe headache followed by a bilateral loss of visual acuity and truncal ataxia. Magnetic resonance imaging revealed abnormal contrast enhancement of both the uveas and the cerebellar vermis corresponding to his neurological abnormalities. The distribution and the nature of the resolution of this unusual pattern of contrast enhancement suggested that these MRI findings might illustrate transient destruction of the blood brain barrier or vascular extravasations. Such events might be representative of pathophysiology involving the central nervous system that occurred in patients with Vogt-Koyanagi-Harada disease.

Cerebellar Ataxia↗
PubMed2005

Supervised learning of postural tasks in patients with poststroke hemiparesis, Parkinson's disease or cerebellar ataxia.

Supervised learning of different postural tasks in patients with lesions of the motor cortex or pyramidal system (poststroke hemiparesis: 20 patients), nigro-striatal system (Parkinson's disease: 33 patients) and cerebellum (spinocerebellar ataxia: 37 patients) was studied. A control group consisted of 13 healthy subjects. The subjects stood on a force platform and were trained to change the position of the center of pressure (CP) presented as a cursor on a monitor screen in front of the patient. Subjects were instructed to align the CP with the target and then move the target by shifting the CP in the indicated direction. Two different tasks were used. In "Balls", the target (a ball) position varied randomly, so the subject learned a general strategy of voluntary CP control. In "Bricks", the subject had to always move the target in a single direction (downward) from the top to the bottom of the screen, so that a precise postural coordination had to be learned. The training consisted of 10 sessions for each task. The number of correctly performed trials for a session (2 min for each task) was scored. The voluntary control of the CP position was initially impaired in all groups of patients in both tasks. In "Balls", there were no differences between the groups of the patients on the first day. The learning course was somewhat better in hemiparetic patients than in the other groups. In "Bricks", the initial deficit was greater in the groups of parkinsonian and cerebellar patients than in hemiparetic patients. However, learning was more efficient in parkinsonian than in hemiparetic and cerebellar patients. After 10 days of training, the hemiparetic and cerebellar patients completed the acquisition at a certain level whereas the parkinsonian patients showed the ability for further improvement. The results suggest that motor cortex, cerebellum, and basal ganglia are involved in voluntary control of posture and learning different postural tasks. However, these structures play different roles in postural control and learning: basal ganglia are mainly involved in learning a general strategy of CP control while the function of the motor cortex chiefly concerns learning a specific CP trajectory. The cerebellum is involved in both kinds of learning.

Adult↗
PubMed1999

Multiple spinal "miliary" hemangioblastomas in von Hippel-Lindau (vHL) disease without cerebellar involvement. A case report and review of the literature.

We report on a 57-year-old male presenting with radicular pain in the nerve roots of L5 and S1 on the right side and dysuria. Magnetic resonance imaging (MRI) of the lumbar spine showed multiple (up to 20) small, intradural enhancing nodules attached to the cauda equina down to the sacrum, the largest 1 cm in diameter at the level Th12/L1 compressing the conus. Additionally, small nodules in the cervico-thoracal region adjacent to the cord, but no cerebellar or cerebral abnormalities, were detected in a consecutive MRI of the remaining neuroaxis. The histology of a resected lesion at Th12/L1 revealed hemangioblastoma of the reticular type. Together with a history of left eye enucleation performed 17 years ago for angiomatosis of the retina and the immunohistochemical detection of von Hippel-Lindau (vHL) protein within the removed spinal hemangioblastoma, a diagnosis of vHL disease was established. Family history and screening for visceral manifestations of vHL disease were negative. In contrast to cerebellar or solitary spinal hemangioblastomas, multiple spinal hemangioblastomas without cerebellar involvement in vHL represent unusual manifestations. Unlike the case for solitary lesions in non-syndromic patients, a surgical cure does not seem feasible in this case. The role of treatment modalities is discussed.

Biomarkers, Tumor↗
PubMed2000

Classically conditioned withdrawal reflex in cerebellar patients. 2. Impaired unconditioned responses.

The study addresses the issue of the role of the cerebellum in human withdrawal-reflex conditioning by comparing data from patients with pure cerebellar diseases (CBL, n = 10) and from cerebellar patients showing additional extracerebellar symptoms (CBL+, n = 10) with those from 11 control subjects (CTRL). During recording sessions, the standard delay-conditioning paradigm with paired-trials was used with tone as the conditioned stimulus (CS). Parameters of the conditioned muscle responses are analyzed in an accompanying paper. Here, we focus on the unconditioned muscle response. A train of current pulses (unconditioned stimulus, US) evoked a lower-limb withdrawal reflex (unconditioned response, UR), which was recorded electromyographically from leg muscles. During the recording sessions with CTRL subjects, UR amplitudes decayed from initially 100% to approximately 50% at the end of the session. This type of decay was clearly less pronounced in the CBL group and minimal in the CBL+ group. Furthermore, the CBL group exhibited UR onsets that were delayed by 20 ms compared with those from CTRL subjects. Although the ranges of measurements characterizing the URs of a given cerebellar patient tested in the paired-trial paradigm overlapped with those of control subjects, the statistically significant differences observed at the group level suggest deficits in the performance of the reflex responses. The delayed URs in patients and the different type of decay of UR amplitudes in repetitively evoked withdrawal reflexes constitute evidence that the cerebellum is critically involved in the control of these UR parameters.

Adult↗
PubMed1980

Romberg and his test.

In the eponymous test described by Romberg in 1846, the erect patient is asked to close his eyes. If he falls, the test is positive and indicates the presence of a dorsal column lesion. Slight modifications have been described in the test but in all cases, great care must be taken to do it carefully. The test compares the stability of eye-opening with that of eye-closure. A positive test does not indicate vestibular or cerebellar disease.

Cerebellar Diseases↗
PubMed1998

A reversible neuronal antibody (anti-Tr) associated paraneoplastic cerebellar degeneration in Hodgkin's disease.

Paraneoplastic cerebellar degeneration (PCD) has been associated with a variety of neoplasms, most commonly with gynecologic tumors, breast cancer, small cell lung cancer, and Hodgkin's disease (HD). In some patients PCD is associated with circulating antineuronal antibodies like anti-Hu, anti-Yo or anti-Ri. Previously, only 5 patients with a new antineuronal antibody called anti-Tr, proposed to be specific for HD, have been reported. We describe 1 further patient with HD and reversible PCD with a decline in anti-Tr antibody titers in cerebrospinal fluid and serum corresponding to the improvement of clinical symptoms. At the present time the immunoreactive pattern observed in rat cerebellum is the only way to identify anti-Tr antibodies and differentiate them from other antibodies that immunoreact with the Purkinje cells.

Adult↗