[Neurology--more than 100 years old and not yet grown up?].
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Biomedical subjects
Publications and source records attributed to T Brandt.
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A retrospective study was conducted on 90 patients with episodic vertigo that could be related to migraine as the most probable pathomechanism. Since the majority of the patients did not fulfill the criteria of the International Headache Society (IHS) for basilar migraine, the diagnosis was substantiated by disease course, medical efficacy in treating (ergotamines) and preventing (metoprolol, flunarizine) attacks, ocular motor abnormalities in the symptom-free interval, and careful exclusion of the most relevant differential diagnoses, such as transient ischemic attacks, Menière's disease, and vestibular paroxysmia. The following clinical features were elaborated. The initial manifestation could occur at any time throughout life, with a peak in the fourth decade in men and a "plateau" between the third and fifth decades in women. The duration of rotational (78%) and/or to-and-fro vertigo (38%) could last from a few seconds to several hours or, less frequently, even days; duration of a few minutes or of several hours was most frequent. Monosymptomatic audiovestibular attacks (78%) occurred as vertigo associated with auditory symptoms in only 16%. Vertigo was not associated with headache in 32% of the patients. In the symptom-free interval 66% of the patients showed mild central ocular motor signs such as vertical (48%) and/or horizontal (22%) saccadic pursuit, gaze-evoked nystagmus (27%), moderate positional nystagmus (11%), and spontaneous nystagmus (11%). Combinations with other forms of migraine were found in 52%. Thus, migraine is a relevant differential diagnosis for episodic vertigo. According to the criteria of the IHS, only 7.8% of these patients would be diagnosed as having basilar migraine. However, to ensure that at least those presenting with monosymptomatic episodic vertigo (78% in our study) receive effective treatment, we propose the use of the more appropriate term "vestibular migraine."
PURPOSE: Comparison of different MR-examination techniques for the diagnosis of acute spontaneous internal carotid artery dissection. PATIENTS AND METHODS: 13 patients (age range 23-59 years) with symptomatic spontaneous dissection of the internal carotid artery were examined. The MRI protocol contained a transverse spin echo sequence, a time-of-flight MR-angiography and a coronal fat suppressed T1-weighted sequence. The earliest examination was performed three days after symptom onset. Follow-up extended up to 30 months. We compared the three different sequences to find out the one that demonstrated the hematoma best. RESULTS: MR-angiography shows a narrowing of the vessel diameter in early examinations. During the subacute stage methemoglobin can obscure this finding. From the third day on fat suppressed T1-weighted images showed a hyperintense hematoma that strongly contrasted to the surrounding fatty tissue. Fat suppressed images showed a hyperintense hematoma up to 10 months after symptom onset while MRA and spin echo sequences did not. CONCLUSIONS: Fat suppressed T1-weighted images are superior in showing vessel wall hematoma and should thus be used in the standard MR-protocol for spontaneous internal carotid artery dissection.
Head positioning can lead to pathological nystagmus and vertigo. In most instances the cause is a peripheral vestibular disorder, as in benign paroxysmal positioning vertigo (BPPV). Central lesions can lead to positional nystagmus (central PN) or to paroxysmal positioning nystagmus and vertigo (central PPV). Lesions in central PPV are often found dorsolateral to the fourth ventricle or in the dorsal vermis. This localization, together with other clinical features (associated cerebellar and oculomotor signs), generally allows one to easily distinguish central PPV from BPPV. However, in individual cases this may prove difficult, since the two syndromes share many features. Even if only BPPV as a peripheral lesion is considered, differentiation based on such features as latency, course, and duration of nystagmus during an attack, fatigability, vertigo, vomiting, and time period during which nystagmus bouts occur, may be impossible. Only the direction of nystagmus during an attack can allow differentiation.
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A unique case of initially right sided varicella zoster induced Ramsay-Hunt syndrome with complete vestibular loss is reported. The patient subsequently developed deficits of the left vestibule 5 months later. An autoimmune pathogenesis of the left vestibular failure rather than bilateral varicella zoster infection was suggested by the following data: (1) no evidence of vesicular eruptions on the left auricle and the virtual absence of antiviral antibodies after onset of bilateral vestibulopathy; (2) prompt response of the left vestibule to immunosuppressive therapy with corticosteroids; and (3) presence of atypical nervous tissue specific autoantibodies against a 45 kDa protein.
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BACKGROUND AND PURPOSE: Both Doppler sonography (DS) and spiral CT angiography (CTA) are noninvasive vascular assessment tools with a high potential for application in acute cerebral ischemia. The usefulness of CTA for vascular diagnosis in acute basilar artery (BA) ischemia has not yet been studied. METHODS: We prospectively studied 19 patients (mean+/-SD age, 58+/-11 years) with clinically suspected acute BA occlusion by DS and CTA. Prior extracranial and transcranial DS was performed in all but 1 patient, with DS 4 hours after CTA. In 6 of 19 patients, we performed digital subtraction angiography. RESULTS: CTA was diagnostic in all but 1 patient. CTA revealed complete BA occlusion in 9 patients and incomplete BA occlusion with some residual flow in 2 patients. A patent BA was shown in 7 patients. Because of severe BA calcification, CTA results were inconclusive in 1 patient. DS was diagnostic in only 7 of 19 patients, indicating certain BA occlusion in 3 patients and BA patency in 4 patients. In an additional 9 patients, the results of DS were inconclusive. DS was false-negative in 2 patients with distal BA occlusion shown by CTA and digital subtraction angiography. In 1 patient with DS performed after CTA, recanalization was demonstrated. In addition to the diagnosis or exclusion of BA occlusion, CTA provided information on the exact site and length of BA occlusion and collateral pathways. In our series, CTA results prompted indication for intra-arterial thrombolysis in 5 patients. CONCLUSIONS: CTA was superior to DS in the assessment of BA patency in patients with the syndrome of acute BA ischemia in terms of feasibility and conclusiveness, particularly in cases with distal BA occlusion. Our study confirmed the usefulness of combined extracranial and transcranial DS in the diagnosis and exclusion of proximal BA occlusion.
BACKGROUND AND PURPOSE: The dermal connective tissue of most patients with spontaneous cervical artery dissections (sCAD) contains abnormal collagen fibers. This suggests a predisposing connective tissue defect. The ultrastructural abnormalities in the skin of patients with sCAD have similarity with the morphological alterations in patients with Ehlers-Danlos syndrome type II, a dominant hereditary disorder that has been correlated in some patients to mutations within the genes encoding type V collagen. The aim of this study was to assess the alpha 1 chain of type V collagen (COL5A1) as a candidate gene for sCAD. METHODS: We searched for mutations in the COL5A1 gene in cDNA from cultured fibroblasts of 19 patients with sCAD using single-strand conformational polymorphism analysis and nucleotide sequence analysis of polymerase chain reaction-amplified fragments of the whole COL5A1 coding sequence. RESULTS: We detected 1 missense mutation leading to a predicted amino acid (192D/N) substitution within the N-terminal propeptide in 2 siblings. All other patients showed regular COL5A1 sequences with some silent polymorphisms. CONCLUSIONS: Mutations in the COL5A1 gene do not appear to be a major factor in the etiology of sCAD.
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BACKGROUND AND PURPOSE: Recently, the diagnostic and therapeutic importance of repeat radiologic imaging in stroke patients has been questioned. The aim of this study was to show the therapeutic and diagnostic consequences of both repeat brain imaging and follow-up vascular imaging in this group of patients. METHODS: Neuroradiologic images and reports as well as clinical records of 317 patients (209 men and 108 women; mean age, 63 years) were reviewed retrospectively to determine the number of modifications made to the diagnosis and therapeutic regimen and to the classification of neuroradiologic findings. RESULTS: Two hundred thirty-eight repeat imaging procedures were performed in 171 patients. Of these, 76 were vascular imaging examinations (11 CT angiograms, 13 MR angiograms, 52 digital subtraction angiograms) and 162 were cross-sectional brain imaging studies (54 MR images, 108 CT scans). Forty of the 76 vascular imaging procedures and 77 of the 162 repeat cross-sectional brain imaging studies led to important diagnostic modifications with consequences for the patients' therapy and prognosis. CONCLUSION: Our study establishes that vascular imaging methods as well as cross-sectional brain imaging used as repeat imaging procedures in stroke patients can have important diagnostic and therapeutic consequences. We believe that repeat imaging in selected subgroups will be cost-effective.
Static vestibulo-ocular brain stem syndromes characterized by skew deviation, a vertical disconjugacy of the eyes, and ocular torsion are the result of a vestibular tone imbalance in the frontal (roll) plane. Similar physiological changes in static eye position, ocular counter-roll and conjugated deviations of vertical eye position, are caused by the influence of gravity mediated by the utricles. These observations prompted our approach with the model described here: based on the known deviations of static eye position, we devised a three-dimensional mathematical model of otolith-ocular function including detailed brain stem anatomy. This model is able to explain and predict the differential effects of unilateral and bilateral peripheral or central vestibular lesions on static eye position in roll, pitch, and yaw planes.
Viral reactivation in temporal ganglia is the suspected cause of Bell's palsy, vestibular neuritis and sudden hearing loss. Since the distribution of latent herpes simplex type 1 (HSV-1) in geniculate, vestibular and spiral ganglia of individual human temporal bones could have implications for the explanation of isolated as well as combined disorders of these three cranial nerves, we examined these ganglia in 18 human temporal bones of adults by nested polymerase chain reaction. In all of the temporal bones HSV-1 specific DNA was detected: 10/18 (56%) of the geniculate, 11/18 (61%) of the vestibular and 9/18 (50%) of the spiral ganglia samples were positive. All combinations of positive and negative ganglia were found in individual temporal bones at roughly equal frequencies. These data support a viral etiology of all three conditions, especially their occasional combinations.
Visual motion perception of a single object, moving with a constant angular velocity of 40 min of arc/s in four orthogonal directions, was measured in eight patients with chronic bilateral vestibular failure (BVF) with the head stationary. Perception of object motion was more severely impaired for horizontal than for vertical directions and the impairment was more pronounced in the dominant eye than the nondominant eye. Impaired motion perception in patients with BVF is best explained by a central visual mechanism that suppresses oscillopsia due to the involuntary retinal slip caused by the defective vestibulo-ocular reflex (VOR). This mechanism cannot be switched off with the head stationary (inactive VOR) and thus causes a measurable deficit of motion perception.