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Sensory and motor responses to deep brain stimulation. Correlation with anatomical structures.

Motor and sensory responses induced by trial stimulation were examined before stereotaxically implanting a permanent stimulating electrode for pain relief in 11 patients with intractable pain of central origin. The total number of points eliciting a response when stimulated was 70. The points of stimulation were determined as exactly as possible from Schaltenbrand and Bailey's Atlas. Motor responses were detected upon stimulating 21 points, the majority of which were in the posterior third of the posterior limb of the internal capsule (IC). Stimulation of these 21 points was accompanied by pain relief in only two points (10%). Warm (22) or cool sensations (three) were provoked in the most posteromedial portion of the posterior limb of the IC, nucleus reticularis pulvinaris, and area triangularis, and seven (28%) of these 25 sensations were accompanied by pain relief. A burning sensation was found upon stimulation of 12 points, with stimulation in the mesencephalic lateral tegmental field eliciting the most severe burning pain. A tingling sensation was elicited at 12 points, in a distribution similar to that of the warm sensation. Five (42%) of these 12 points provided pain relief. The best stimulating point for pain relief is not in the center of the posterior limb of the IC, directly lateral to the posterior commissure, but rather in its most posteromedial part; that is, at the nucleus reticularis pulvinaris or area triangularis.

Adult↗

Cerebral effects of isovolemic hemodilution with a hypertonic saline solution.

In view of a growing interest in the resuscitative use of hypertonic saline solutions, the authors have examined the cerebral effects of isovolemic hemodilution carried out over 1 hour (hematocrit decreased from 40% to 20%, stable arterial and right arterial pressures), using a hypertonic lactated Ringer's solution (HT-LR: Na+ 252 mEq/liter, osmolality 480 mOsm/liter). Experiments were carried out in anesthetized ventilated rabbits. Measured variables included cerebral blood flow (using the H2 clearance method), intracranial pressure (ICP), the electroencephalogram, spinal cord and skeletal muscle water content (%H2O), and the specific gravity of small (10- to 30-mg) tissue samples taken from different areas of the left hemisphere (including the cortex, thalamus, internal capsule, and hippocampus). The changes produced by HT-LR were compared with those seen in both undiluted control animals and in rabbits hemodiluted with normal saline (Na+ 155 mEq/liter, osmolality 310 mOsm/liter). The results demonstrate that hemodilution with HT-LR leads to the expected increases in serum Na+ and osmolality (158 +/- 6 mEq/liter and 320 +/- 5 mOsm/kg, respectively, mean +/- standard deviation) and that these were accompanied by reductions in the %H2O of all cerebral and extracerebral tissues, increases in the specific gravity of all tissue regions studied, and a decrease in ICP (1.9 +/- 0.7 mm Hg). By contrast, rabbits with hemodilution by normal saline showed no changes in either %H2O or specific gravity, but had significant increases in ICP (3.3 +/- 1.3 mm Hg). Cerebral blood flow increased in all animals hemodiluted with either HT-LR or normal saline by a combined average of +29 ml/100 gm/min. Although these studies were performed in neurologically normal animals, the combination of cerebral changes seen with HT-LR (cerebral dehydration, less peripheral edema, decreased ICP but with increased cerebral blood flow) suggests that this approach may have some advantages over the use of isotonic fluids, and may have some utility in the resuscitation of head-injured patients.

Animals↗

Variability of the territories of the major cerebral arteries.

Recent morphological and functional studies on the circle of Willis suggest that the areas of supply of the six major cerebral arteries show a considerable variation in distribution, in contrast to the relatively consistent pattern generally accepted; therefore, the cortical and intracerebral distribution of the territories of these arteries was investigated in 25 unfixed human brains obtained at routine autopsy. The six major cerebral arteries were simultaneously injected under the same pressure with different-colored Araldite F mixtures under standardized conditions to obtain the most realistic territorial distribution. The cortical boundaries were examined and recorded in relation to the cerebral gyri and sulci, and the territories of the anterior, middle, and posterior cerebral arteries were analyzed and compared. The intracerebral distribution of these territories was investigated after the injected brains were cut in parallel slices. The variability of the territories of these arteries was much larger than generally described in the literature. Twenty-six variations in the territory of the anterior cerebral artery, 17 variations in the area of the middle cerebral artery, and 22 variations in the area of the posterior cerebral artery were found in the cortex of 50 hemispheres. Intracerebrally, the anterior, middle, and posterior cerebral arteries contributed in varying degrees to the blood supply of the lobar white matter, the internal capsule, the caudate nucleus, and the lentiform nucleus. The large variation in the area in which the cortical and intracerebral boundaries between these territories was located was demonstrated by illustrating the minimum and maximum extent of each. The results are compared with prior findings, and their implications for both experimental model studies and clinical practice are discussed.

Adolescent↗

A new model of diffuse brain injury in rats. Part II: Morphological characterization.

A new model producing diffuse brain injury, without focal brain lesions, has been developed in rats. This has been achieved by allowing a weight of 450 gm to fall onto a metallic disc fixed to the intact skull of the animal which is supported by a foam bed. Two levels of injury were examined by adjusting the height of the falling weight to either 1 m or 2 m. Two groups of animals were studied. Group 1 animals were separated into three subgroups: 10 received a 1-m weight drop, 58 received a 2-m weight drop, and 13 served as controls; all were allowed to breathe spontaneously. Group 2 animals were separated into the same subgroups: four received a 1-m weight drop, six received a 2-m weight drop, and four served as controls; all of these were mechanically ventilated during the procedure. In Group 1, morphological studies using light and electron microscopy were performed at 1, 6, 24, or 72 hours, or 10 days after insult; all Group 2 rats were studied at 24 hours after injury. Results from Group 1 animals showed that no mortality occurred with the 1-m level injury, while 59% mortality was seen with the 2-m level injury. On the other hand, no mortality occurred in Group 2 animals regardless of the level of trauma induced. However, the morphological changes observed in both groups were similar. Gross pathological examination did not reveal any supratentorial focal brain lesion regardless of the severity of the trauma. Petechial hemorrhages were noticed in the brain stem at the 2-m level injury. Microscopically, the model produced a graded widespread injury of the neurons, axons, and microvasculature. Neuronal injury was mainly observed bilaterally in the cerebral cortex. Brain edema, in the form of pericapillary astrocytic swelling, was also noted in these areas of the cerebral cortex and in the brain stem. Most importantly, the trauma resulted in a massive diffuse axonal injury that primarily involved the corpus callosum, internal capsule, optic tracts, cerebral and cerebellar peduncles, and the long tracts in the brain stem. It is concluded that this model would be suitable for studying neuronal, axonal, and vascular changes associated with diffuse brain injury.

Animals↗

Methods for microelectrode-guided posteroventral pallidotomy.

Methods for localizing the posteroventral globus pallidus intermus are described. The authors' techniques include the use of microelectrodes to record single-unit activity and to microstimulate in human pallidum and its surrounding structures. This technique allows a precise determination of the locations of characteristic cell types in sequential trajectories through the external and internal segments of the pallidum. The location of the optic tract can be determined from microstimulation-evoked visual sensations and recordings of flash-evoked potentials. In addition, microstimulation-evoked motor and sensory responses allow the internal capsule to be identified. The data collected using this technique are an important adjunct to selecting optimum sites to place electrocoagulation lesions for stereotactic posteroventral pallidotomy for refractory Parkinson's disease.

Catheter Ablation↗

Microelectrode-guided posteroventral pallidotomy for treatment of Parkinson's disease: postoperative magnetic resonance imaging analysis.

The authors report the postoperative magnetic resonance (MR) imaging findings in 36 patients with advanced Parkinson's disease who underwent unilateral microelectrode-guided posteroventral pallidotomy. The lesions were placed within 1 mm of the ventral border of the globus pallidus internus (GPi) to include pallidothalamic outflow pathways. Sequential MR studies were obtained within 1 to 3 days postoperatively and at 6-month follow-up examination. Thirty-four (94%) of the 36 patients enjoyed sustained moderate or marked improvement of their parkinsonian symptoms 6 months postoperatively. Transient side effects occurred in five patients (14%), but there were no persistent complications. The pallidal radiofrequency lesions were prolate spheroid shaped and were composed of three concentric zones in the early postoperative studies. The mean volume of the middle zone, corresponding to the area of hemorrhagic coagulation necrosis, was 44.4 +/- 17.6 mm3; the mean lesion volume as defined by the outer zone, corresponding to perilesional edema, was 262.2 +/- 111.6 mm3. Additional edema spreading to the internal capsule was noted in 32 of 34 cases and to the optic tract in 11 of 34 cases. In two patients small ischemic infarctions involving the corona radiata were found, and in one a venous infarction was detected. Ischemic infarction resulted in mild transient Broca's aphasia in one patient, but there was no detectable neurological deficit in the other two. The mean volume of late-phase (6 months) lesions was 22 +/- 28.8 mm3. In three patients no lesion was identified despite sustained clinical improvement. The lesion was located in the posteroventral GPi in all cases except in one patient in whom it was confined to the GP externus (GPe). This 49-year-old woman did not experience sustained benefit. The authors found no consistent correlations between lesion size and location and clinical outcome as measured by a global outcome score, the Unified Parkinson's Disease Rating Scale motor, activities of daily living, and bradykinesia "off" scores or rating of dyskinesias. Lesioning of pallidal and subpallidal pathways may contribute to the sustained clinical benefit in this series. Magnetic resonance imaging analysis showed that intraoperative microelectrode recording facilitated accurate placement of the lesion in this critical area.

Adult↗

Germinoma causing wallerian degeneration. Case report and review of the literature.

Germinomas occurring in the thalamus and basal ganglia sometimes cause atrophy of the cerebral hemisphere on the affected side. The authors present the case of a 12-year-old girl with a germinoma that developed in the basal frontal lobe and cerebral basal ganglia. Magnetic resonance imaging showed atrophy not only of the cerebrum but also of the brainstem. A T2-weighted image revealed an area of high intensity that proved to be wallerian degeneration extending from the corona radiata and internal capsule to the brainstem. The authors suggest that this pathological change may be involved in the development of the symptoms and hemiatrophy associated with germinomas in this region of the brain.

Brain Neoplasms↗

Reversal of experimental parkinsonism by using selective chemical ablation of the medial globus pallidus.

OBJECT: Symptoms from Parkinson's disease improve after surgical ablation of the medial globus pallidus (GPm). Although, in theory, selective chemical ablation of neurons in the GPm could preserve vital structures jeopardized by surgery, the potential of this approach is limited when using traditional techniques of drug delivery. The authors examined the feasibility of convection-enhanced distribution of a neurotoxin by high-flow microinfusion to ablate the neurons of the GPm selectively and reverse experimental Parkinson's disease (akinesia, tremor, and rigidity). METHODS: Initially, to test the feasibility of this approach, the GPms of two naive rhesus macaques were infused with kainic acid or ibotenic acid through two cannulas that had been placed using the magnetic resonance imaging-guided stereotactic technique. Two weeks later the animals were killed and their brains were examined histologically to determine the presence of neurons in the GPm and the integrity of the optic tract and the internal capsule. To examine the therapeutic potential of this paradigm, unilateral experimental Parkinson's disease was induced in six macaques by intracarotid infusion of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) and their behavior was studied for 12 weeks after chemopallidotomy was performed using kainic acid (three animals) or control infusion (three animals). CONCLUSIONS: Chemopallidotomy using kainic acid permanently reversed the stigmata of MPTP-induced parkinsonism. By contrast, the control animals exhibited a transient recovery following intrapallidal infusion and then relapsed back to their baseline state. The use of high-flow microinfusion of selectively active toxins has the potential for treatment of Parkinson's disease and, by expanding the range of approachable targets to include large nuclei, for broad applications in clinical and experimental neuroscience.

Animals↗

Staged bilateral pallidotomy for treatment of Parkinson disease.

OBJECT: Several investigators have described the motor benefits derived from performing unilateral stereotactic pallidotomy for the treatment of Parkinson disease (PD), but little is known about the efficacy and complication rates of bilateral procedures. The goal of this study was to assess both these factors in 12 patients. METHODS: Eleven patients with medically intractable PD underwent staged bilateral pallidotomy and one patient underwent a simultaneous bilateral procedure. Unilateral pallidotomy resulted in an improvement in the patients' Unified Parkinson Disease Rating Scale (UPDRS) total scores and motor subscores, Hoehn and Yahr stages, and Schwab and England Activities of Daily Living scores. There were no complications. The second procedures were performed 5 to 25 months after the first, and nearly complete 3-month follow-up data are available for eight of these patients. Staged bilateral pallidotomy did result in further improvements in some symptoms, but the patients proved to be less responsive to levodopa. In contrast to outcomes of the initial unilateral pallidotomy, there were significant complications. One patient suffered an acute stroke, two patients suffered delayed infarctions of the internal capsule, four patients had mild-to-moderate worsening of speech and increased drooling, and one patient complained of worsening memory. CONCLUSIONS: Bilateral pallidotomy results in modest benefits but is associated with an increased risk of complications.

Adult↗

Anatomical analysis of different hemispherotomy procedures based on dissection of cadaveric brains.

OBJECT: Functional hemispherectomy, itself a modification of anatomical hemispherectomy, has been further modified to a less invasive method (hemispherotomy), in which cortical resection is minimized and the rest of the affected hemisphere is functionally isolated by transecting its projection and commissural fibers. Although descriptions of three different types of hemispherotomy procedures have been published, the authors believe that it is important to develop a common and universally acceptable method based on a systematic analysis of topographic anatomy and neuronal connections. To this end, they have analyzed the three aforementioned procedures on the basis of meticulous fiber dissections in previously frozen formalin-fixed human brains. METHODS: The brain anatomy pertinent to surgical hemispherotomy is described in conjunction with dissection studies in 14 previously frozen, formalin-fixed human brains. The anatomical landmarks necessary for performing particular neuronal fiber resections are identified, and their relationships with operative methods are discussed, with an emphasis on commonalities among the three hemispherotomy procedures. CONCLUSIONS: In this analysis the authors confirmed that hemispherotomy typically consists of four common procedures: 1) interruption of the internal capsule and corona radiata; 2) resection of the medial temporal structures; 3) transventricular corpus callosotomy; and 4) disruption of the frontal horizontal fibers. After meticulous dissection of cadavers, the authors have designated a reliable method for performing these four operations that may be applicable as a commonly used procedure.

Adult↗

Diffusion-tensor imaging-guided tracking of fibers of the pyramidal tract combined with intraoperative cortical stimulation mapping in patients with gliomas.

OBJECT: The goal of this study was to use diffusion-tensor (DT) magnetic resonance (MR) imaging to track fibers combined with cortical stimulation mapping to delineate descending motor pathways. Subcortical localization of motor pathways in relation to a glioma may provide critical information to guide tumor resection and prevent surgical morbidity. METHODS: Eleven adult patients harboring gliomas underwent MR imaging 1 day prior to image-guided intraoperative cortical motor mapping and tumor resection. Screens depicting 27 cortical motor sites on a surgical navigation system were saved to launch DT imaging of fiber tracks of descending motor pathways. The position and organization of motor tracts were visualized by fiber tracking. Tracks from 16 motor stimulation sites followed descending pathways from the precentral gyrus, through the corona radiata and internal capsule, and into the cerebral peduncle. These tracks were also observed on DT images to diverge along crossing white matter bundles (four patients) and to terminate or deviate in regions of peritumoral vasogenic edema (five patients). CONCLUSIONS: The use of precise intraoperative cortical mapping information and DT images of fiber tracks can reveal the course of motor pathways beneath the cortex. The subcortical fiber tracks generated are consistent with the known anatomical course and somatotopic organization of the motor tract in relation to its cortical origins. Tracking fibers by using DT imaging in combination with functional localization has the potential to reduce surgical morbidity by revealing subcortical connections of the functional cortex.

Adult↗

Deep brain stimulation of the ventral caudate nucleus in the treatment of obsessive-compulsive disorder and major depression. Case report.

Obsessive-compulsive disorder (OCD) is an anxiety disorder associated with recurrent intrusive thoughts and repetitive behaviors. Although conventional pharmacological and/or psychological treatments are well established and effective in treating OCD, symptoms remain unchanged in up to 30% of patients. Deep brain stimulation (DBS) of the anterior limb of the internal capsule has recently been proposed as a possible therapeutic alternative in treatment-resistant OCD. In the present study, the authors tested the hypothesis that DBS of the ventral caudate nucleus might be effective in a patient with intractable severe OCD and concomitant major depression. Psychiatric assessment included the Yale-Brown Obsessive Compulsive Scale (Y-BOCS), the Hamilton Depression Rating Scale (HDRS), the Hamilton Anxiety Rating Scale (HARS), and the Global Assessment of Functioning (GAF) Scale for determining the symptom severity of OCD, depression, and anxiety as well as the quality of pychosocial and occupational functioning, respectively. Neuropsychological assessment consisted of a wide range of tests primarily exploring memory and executive functions. Deep brain stimulation of the ventral caudate nucleus markedly improved symptoms of depression and anxiety until their remission, which was achieved at 6 months after the start of stimulation (HDRS < or = 7 and HARS < or = 10). Remission of OCD (Y-BOCS < 16) was also delayed after 12 or 15 months of DBS. The level of functioning pursuant to the GAF scale progressively increased during the 15-month follow-up period. No neuropsychological deterioration was observed, indicating that DBS of the ventral caudate nucleus could be a promising strategy in the treatment of refractory cases of both OCD and major depression.

Caudate Nucleus↗

Anatomical landmarks for hemispherotomy and their clinical application.

OBJECT: The authors introduce the surgical concept of the central core of a hemisphere, from which anatomical structures are disconnected during most current hemispherotomy techniques. They also propose key anatomical landmarks for hemispherotomies that can be used to disconnect the hemisphere from its lateral surface around the insula, through the lateral ventricle toward the midline. METHODS: This anatomical study was performed in five adult cadaveric heads following perfusion of the cerebral arteries and veins with colored latex. Anatomical landmarks were used in five hemispheric deafferentations. The central core of a hemisphere consists of extreme, external, and internal capsules; claustrum; lentiform and caudate nuclei; and thalamus. Externally, this core is covered by the insula and surrounded by the fornix, choroid plexus, and lateral ventricle. During most hemispherotomies, the surgeon reaches the lateral ventricle through the frontoparietal opercula or temporal lobe; removes the mesial temporal structures; and disconnects the frontal lobe ahead, the parietal and occipital lobes behind, and the intraventricular fibers of the corpus callosum above the central core. After a temporal lobectomy, the landmarks include the choroid plexus and posterior/ascending portion of the tentorium to disconnect the parietal and occipital lobes, the callosal sulcus or distal anterior cerebral artery (ACA) to sever the intraventricular fibers of the corpus callosum, and the head of the caudate nucleus and ACA to detach the frontal lobe. CONCLUSIONS: These landmarks can be used in any hemispherotomy during which a cerebral hemisphere is disconnected from its lateral surface. Furthermore, they can be used to perform any resection around the central core of the hemisphere and the tentorial incisura.

Adult↗

Use of sagittal images for localization of the subthalamic nucleus. Technical note.

Direct observation of the subthalamic nucleus (STN) is not always possible, and physicians at many centers rely on indirect methods that relate the position of the STN to more easily recognized structures such as the red nucleus (RN). In this paper the authors describe an indirect method of viewing the STN based on the anatomy depicted on sagittal magnetic resonance (MR) images. A review of sagittal slices appearing in standard stereotactic atlases showed that the STN lies within the angle formed by the descending internal capsule (IC) and the substantia nigra (SN). The authors' technique consists of marking the location of the STN in this nigrocapsular angle on each sagittal MR image between the RN and the lateral border of the brainstem, and transferring these points to axial MR images to build a locus of points used to describe the STN. A point is chosen in the center of this locus as the stereotactic target. Two hundred eighty-two sagittal images obtained from 71 MR imaging studies performed in 29 patients by using a fast-spin echo inversion-recovery technique were examined for the presence of the STN, the SN, and the IC. The descending IC could be detected in 97% of the slices, the SN in 95% of the slices, and the STN in 73% of the slices. This indirect method involving sagittal anatomy can be used to refine localization of the STN.

Humans↗

Neuronal damage in hydrocephalus and its restoration by shunt insertion in experimental hydrocephalus: a study involving the neurofilament-immunostaining method.

OBJECT: The morphological and functional impairments of neurons and their connections caused by hydrocephalus, and their restoration by ventricular shunt placement were investigated in experimental hydrocephalus by the immunostaining of neurofilaments, which constitute the major component of the neuronal cytoskeleton. METHODS: Progressive hydrocephalus was induced in 15 young mongrel dogs 1 to 2 months of age, 3 to 4 weeks after cisternal injection of kaolin. The dogs were divided into three groups of five animals each, a "preshunt," "post-shunt," and "nonshunt" group, depending on whether the hydrocephalic animals underwent a procedure to insert a ventriculoperitoneal shunt. Neurofilament, glial fibrillary acid protein (GFAP), and synaptophysin immunostaining were performed using samples of brain tissue from each hydrocephalic group and a fourth "control" group (five animals). In the cortex, morphological deformation and heterogeneous neurofilament immunoreactivity of the apical dendrites became pronounced in accordance with the progression of hydrocephalus (from the preshunt to the nonshunt group), and these changes remained after shunt insertion (postshunt group). In the periventricular white matter, swollen and fragmented axons increased in number along with hydrocephalic progression and were incompletely repaired by ventricular shunt placement. The GFAP-positive astrocytes observed around repaired axons in the postshunt group were seen more prominently than in the untreated hydrocephalic groups. In the internal capsule, fairly good recovery from axonal damage caused by the hydrocephalic condition was achieved by insertion of a ventricular shunt, compared with that seen in the periventricular white matter. CONCLUSIONS: Cytoskeletal damage of neurons in hydrocephalus and its incomplete restoration by shunt placement were most significant in the periventricular white matter. This finding may account for the impaired cognitive function seen in children who have shunts and an apparently reconstituted cerebral mantle; therefore, neuronal protection in the early hydrocephalic state should be considered.

Age Factors↗

[Neuro-otological examination in patients with unilateral cerebral lesion--fixation suppression].

Fixation suppression of caloric nystagmus was examined in 33 cases with unilateral cerebral vascular lesion. Only Lesion included the unilateral internal capsule was certified by CT scan in all of the subjects. The rate of fixation suppression (FS%): FS% = (1 - F/D) x 100 was calculated by the mean slow-phase velocity (F) of caloric nystagmus during fixation and the maximum slow-phase velocity (D) during eyes open in darkness. The normal FS% of nystagmus to both sides was observed in 12 cases, the reduction of FS% of nystagmus to the ipsilateral side of the lesion was observed in 11 cases, that of nystagmus to the contralateral side in one, and that of nystagmus to the bilateral side in eight. In 30 out of 32 cases which were able to measure the slow-phase velocity (SPV) of caloric nystagmus, FS% of nystagmus to the ipsilateral side of the lesion was lower than that to the contralateral side, and there was statistically a significant difference between them (p less than 0.05). Furthermore, the difference between the SPV of nystagmus to the ipsilateral side of the lesion and that to contralateral side was examined during fixation and during eyes open in darkness separately. The SPV of nystagmus to the ipsilateral side of the lesion was significantly (p less than 0.01) higher than that to the contralateral side during fixation. There was no significant difference between them during eyes open in darkness. In 15 cases, however, tonic eye deviation over 20 degrees to the slow-phase side was only observed during nystagmus to the ipsilateral side.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Isolated dysarthria-facial paresis syndrome: a rare clinical entity which is usually overlooked.

BACKGROUND AND AIMS: The aim of the study is to search the lesion localization of the pure isolated facial paresis-dysarthria syndrome in patients who were admitted to our neurology clinic in a prospective study. METHODS: Over a period of six years, the patients who had no prominent sensorimotor dysfunction were examined by neurologists and underwent computerized tomography (CT) and/or magnetic resonance imaging (MRI). RESULTS: Eleven patients out of more than 2000 had the aforementioned clinical picture. Lacunar infarctions were identified at the corona radiata in nine patients, and at the internal capsule in two patients. As reported previously, facial paresis was usually mild and temporary. Six of our eleven patients were seen at the outpatient clinic one month later. Four of them had completely recovered and the other two had mild dsyarthria without any facial paresis. The other five could not be reached after leaving the hospital. CONCLUSIONS: Dysarthria-facial paresis is a rare clinical entity and possibly a variation of dysarthria-clumsy hand syndrome, and we suggest that pure facial paresis (FP) and pure dysarthria should be considered as very extreme examples of this syndrome.

Aged↗

[Evaluation of brain in myotonic dystrophy using diffusion tensor MR imaging].

In many cases of myotonic dystrophy, high-intensity areas are seen in the cerebral white matter on T2-weighted imaging. Brain MRI was performed in 15 patients with myotonic dystrophy using diffusion tensor imaging, which is sensitive to the detailed structure of white matter, and the results were compared with those of normal controls. FA (anisotropic diffusion) values in the cerebral white matter of myotonic dystrophy patients were significantly lower than those of normal controls (p< 0.01), even if the hyperintense lesion was not seen on T2-weighted imaging. Values of trace (isotropic diffusion) in myotonic dystrophy patients were significantly higher than those of normal controls (p< 0.05), except in the posterior limb of the internal capsule. Diffusion tensor imaging could detect pathological change of the cerebral white matter in myotonic dystrophy patients, and may be useful for quantification and detection of subtle pathological change.

Adult↗