Trigeminal trophic syndrome: successful treatment with carbamazepine.
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Definitions of classical crossed brainstem syndromes in the modern neurological literature are often inaccurate and inconsistent. As a result, different clinical syndromes are designated with the same eponym, other crossed syndromes are nearly completely forgotten. In this study, the original historical publications on the classical alternating pontine syndromes of Foville, Millard-Gubler, Raymond, Raymond-Cestan, Brissaud-Sicard, Gasperini, Grenet and Gelle were reviewed and critically analysed. Their anatomic basis and etiology, and the main publications about each syndrome were discussed. We conclude that the syndromes of Foville, Millard-Gubler, Raymond, Raymond-Cestan and Brissaud-Sicard are interpreted in their essential parts concurring to their historical descriptions. Crossed syndromes of Foville and Millard-Gubler are occasionally mixed up with each other. The syndromes in the last decades described as crossed syndromes of Gasperini and Grenet were, however, never described by Gasperini and Grenet. The existence of the Gelle's as "paralysie alterné de l'acoustique" postulated syndrome seems to be very questionable.
OBJECTIVE: To describe the disordered swallowing patterns in recurrent cortical/subcortical stroke and first-time brainstem stroke. DESIGN: A retrospective study. SUBJECTS: Forty-seven consecutive patients, 28 with recurrent cortical/subcortical stroke and 19 with first-time brainstem stroke, referred for dysphagic evaluation to the rehabilitation department of a medical centre. METHODS: Thirty-five male and 12 female patients with a mean age of 62.0+/-11.5 years were included. The median post-stroke duration was 17.0 days. The records of clinical examination and a videofluoroscopic study of swallowing were collected through chart review. The percentages of abnormalities seen at clinical examination and videofluoroscopic swallowing study between recurrent cortical/subcortical stroke and first-time brainstem stroke patients were compared using a chi-square test. RESULTS: The recurrent cortical/subcortical patients suffered from a higher rate of impaired tongue movement, drooling and aphasia at clinical examination and a higher percentage of swallowing abnormalities in oral-preparatory and oral phases in the videofluoroscopic swallowing study. The abnormal videofluoroscopic findings in first-time brainstem stroke patients predominantly occurred in the pharyngeal phase. Both groups had more difficulties swallowing thin barium than they did swallowing the thick and paste barium. CONCLUSION: The recurrent cortical/subcortical stroke and first-time brainstem stroke patients show different manifestations in some parameters of both clinical examination and videofluoroscopic swallowing study.
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL) is a hereditary arteriopathy caused by mutations of the Notch3 gene. The risk factors for cerebral microhaemorrhages (CM), their relationship to other MRI lesions in the disease and their potential clinical impact have not been previously defined. Our purpose was to examine the frequency, number and location of microhaemorrhages in a multicentre cohort study, defining predisposing factors and associated radiographic markers in CADASIL patients. We collected clinical data from 147 consecutive patients enrolled in an ongoing prospective cohort study. Degree of neurological disability and cognitive impairment were assessed by standardized scales. T(1)-weighted, FLAIR and T2*-weighted gradient-echo (GE) MRI sequences were performed. Volume and location of lacunar infarcts and white matter hyperintensity (WMH) were assessed. Number and location of CM were recorded. CM were present in 35% patients, most commonly occurring in the thalamus, brainstem and basal ganglia. The location of CM qualitatively differed from areas of lacunar infarction and WMH. There was a significant association between the presence of CM and a history of hypertension (P = 0.005), systolic blood pressure (SBP) (P = 0.014), haemoglobin A1c (HbA1c) (P = 0.004) and the volume of lacunar infarcts (P = 0.010) and WMHs (P = 0.046). The number of CM was independently associated with SBP (P = 0.005), the diagnosis of hypertension (P = 0.0004), volume of WMH (P = 0.0005) and lacunar infarcts (P = 0.004). In contrast, no association was found between blood pressure or HbA1c and the load of WMH or lacunar infarcts. The presence of CM was independently associated with increased modified Rankin scores. CM are independently associated with blood pressure and HbA1c as well as with lacunar infarct and WMH volume in CADASIL. Both the vascular risk factors and regional distribution of CM appear distinct from those associated with other MRI markers, suggesting a distinct pathological process. These lesions have a potential clinical impact in CADASIL. These findings further suggest that modulation of blood pressure and glucose levels might influence the course of the disease.
OBJECTIVE: This study was conducted to delineate the incidence and outcome of dysphagia among hospitalized patients who were referred for rehabilitation because of brainstem stroke. DESIGN: We retrospectively reviewed the medical records of 36 patients who were admitted because of brainstem stroke. Information on the patients' clinical features, feeding status, and the results of clinical and videofluoroscopic swallowing examinations were obtained through chart review. Follow-up interviews were conducted via telephone to learn the general medical condition and feeding status of the patients 7-43 mo after hospital discharge. RESULTS: A total of 81% of the patients had dysphagia at the time of initial clinical swallowing evaluation, which was performed 10-75 days after the onset of stroke. A total of 79% of the dysphagic individuals depended on tube feeding at the initial evaluation; 22% of all individuals could not resume oral intake at discharge. Statistical analyses revealed a significant association between poor outcome and disease involving the medulla, the presence of a wet voice during the initial swallowing test, and a delay or absence of the swallowing reflex. The incidence of aspiration pneumonia was 11%. There was a correlation between the detection of aspiration by modified barium meal videofluoroscopy and the development of aspiration pneumonia. Follow-up interviews showed that 88% of the 27 patients who were contacted had resumed full oral intake 4 mo after the onset of stroke. CONCLUSIONS: The incidence of dysphagia was relatively high in our study population. The long-term outcome was favorable.
We describe a patient with locked-in syndrome who had minimal volitional motor function and severe spasticity in all four extremities. The patient showed a significant improvement in volitional motor function following intrathecal baclofen pump therapy to control spasticity. This case study suggests that intrathecal baclofen pump therapy might improve motor function in select patients with locked-in syndrome.
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A 78-year-old man suffered from refractory aspiration pneumonia as a result of a minor medullary stroke. The only neurological symptom observed in this patient was difficulty in swallowing. He was managed with i.v. hyperalimentation with termination of oral intake, including water. However, he still experienced several episodes of aspiration pneumonia. As he was considered to have a bacterial infection because of silent aspiration of colonized oropharyngeal material, inhalation of tobramycin was introduced and successful control of airway infection was attained.
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OBJECTIVES: To analyze the causes and long-term clinical outcome of internuclear ophthalmoplegia (INO) in a consecutive series of 65 patients. PATIENTS AND METHODS: From a clinical registry of a neuroophthalmological department, patients with diagnosis of INO were retrospectively identified. Patients were classified into three groups: unilateral INO, bilateral INO, and one-and-a-half syndrome. RESULTS AND CONCLUSIONS: Thirty-three men (50.8%) and 32 women (49.2%), with a mean age of 38.4 years were included in the study. INO was unilateral in 36 patients (55.4%), bilateral in 22 (33.8%) and one-and-a-half syndrome in seven (10.8%). The most common causes were vascular (36.9%), multiple sclerosis (32.3%), and infectious diseases (13.8%). Resolution of INO was documented in 32 patients (49.2%): 15 patients showed INO resolution during the first 3 months and 17 patients in 3-9 months. INO persisted in 33 patients (50.8%) even after a follow-up longer than 12 months.
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OBJECTIVE: Drug resistant neurogenic pain can be relieved by repetitive transcranial magnetic stimulation (rTMS) of the motor cortex. This study was designed to assess the influence of pain origin, pain site, and sensory loss on rTMS efficacy. PATIENTS AND METHODS: Sixty right handed patients were included, suffering from intractable pain secondary to one of the following types of lesion: thalamic stroke, brainstem stroke, spinal cord lesion, brachial plexus lesion, or trigeminal nerve lesion. The pain predominated unilaterally in the face, the upper limb, or the lower limb. The thermal sensory thresholds were measured within the painful zone and were found to be highly or moderately elevated. Finally, the pain level was scored on a visual analogue scale before and after a 20 minute session of "real" or "sham" 10 Hz rTMS over the side of the motor cortex corresponding to the hand on the painful side, even if the pain was not experienced in the hand itself. RESULTS: and discussion: The percentage pain reduction was significantly greater following real than sham rTMS (-22.9% v -7.8%, p = 0.0002), confirming that motor cortex rTMS was able to induce antalgic effects. These effects were significantly influenced by the origin and the site of pain. For pain origin, results were worse in patients with brainstem stroke, whatever the site of pain. This was consistent with a descending modulation within the brainstem, triggered by the motor corticothalamic output. For pain site, better results were obtained for facial pain, although stimulation was targeted on the hand cortical area. Thus, in contrast to implanted stimulation, the target for rTMS procedure in pain control may not be the area corresponding to the painful zone but an adjacent one. Across representation plasticity of cortical areas resulting from deafferentation could explain this discrepancy. Finally, the degree of sensory loss did not interfere with pain origin or pain site regarding rTMS effects. CONCLUSION: Motor cortex rTMS was found to result in a significant but transient relief of chronic pain, influenced by pain origin and pain site. These parameters should be taken into account in any further study of rTMS application in chronic pain control.
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OBJECTIVE: To determine the clinical presentation and aetiology of small subcortical infarctions as found on diffusion weighted magnetic resonance imaging (DWI). DWI is both sensitive and specific in the early detection of acute ischaemic brain lesions irrespective of pre-existing vascular damage. METHODS: Ninety three patients were identified showing subcortical or brainstem DWI lesions <1.5 cm in diameter within a maximum of 7 days from the onset of stroke symptoms. The patients' clinical status on admission was reviewed according to the Oxfordshire Community Stroke Project (OCSP). The results of procedures searching for cerebrovascular risk factors, large artery disease, and potential sources of cardiac embolism were included to determine stroke aetiology. Magnetic resonance imaging scans were also reviewed for concomitant changes that could support the aetiologic classification. RESULTS: Only 41 (44.1%) patients presented clinically with a lacunar syndrome according to OCSP criteria. The nine (9.7%) patients who showed two or more DWI lesions in different vascular territories were also significantly more likely to have potential sources of cardiac embolism (5/9, 55.6% v 20/84, 23.8%). Hypertension was significantly more prevalent in the group of patients who showed a microangiopathy related imaging pattern, but this pattern did not exclude the presence of large artery disease or a possible cardioembolic source of stroke. CONCLUSION: Identification of small subcortical infarctions as the cause of stroke appears quite uncertain based on clinical characteristics only. DWI adds significant aetiologic information but does not obviate the search for other potentially causative mechanisms.
Cognitive dysfunction adversely influences long term outcome after cerebral insult, but the potential for brain stem lesions to produce cognitive as well as physical impairments is not widely recognised. This report describes a series of seven consecutive patients referred to a neurological rehabilitation unit with lesions limited to brain stem structures, all of whom were shown to exhibit deficits in at least one domain of cognition. The practical importance of recognising cognitive dysfunction in this group of patients, and the theoretical significance of the disruption of specific cognitive domains by lesions to distributed neural circuits, are discussed.
The nature of impairment in hand motor control after stroke and its relationship to hand function are still not well understood. In this study, we investigated digit independence in patients with subcortical stroke (n = 8) and moderate hand impairment, defined by wrist and hand Fugl-Meyer scale scores < or =25/33, and age-matched controls (n = 8). Subjects made cyclical flexion-extension movements of an instructed digit while keeping the other digits as still as possible. Movements of the metacarpo-phalangeal (MCP) joints of the five digits were measured using an instrumented glove. The ability to move an instructed digit individually (individuation index), and the ability to keep a noninstructed digit as still as possible (stationarity index) were determined for each digit. Contrary to the finding of normal thumb individuation in a recent study of patients with variable hand motor impairment after stroke, we found that independent movement for all digits was significantly impaired, although individuation and stationarity were differentially affected for each digit. All the digits, including the thumb, showed a similar impairment in individuation. In contrast, stationarity was affected in a digit-dependent pattern: the thumb was affected least, and the middle finger was most impaired. Stroke subjects did not extend their digits fully to the baseline position, and the angular displacement at maximum digit extension correlated significantly with digit individuation. Contrary to expectation, digit independence correlated weakly with clinical tests of hand function, which emphasize grasp. This suggests that corticospinal projections might be separated with respect to function rather than finger topography.