Basilar branch pontine infarction with prominent sensory signs

We identified 10 patients with acute pontine infarction and specific sensory findings. Two patients had pure sensory symptoms, two had sensory complaints of the hand and mouth, and the other six had hemisensory loss referable to medial lemniscal or spinothalamic tract dysfunction but localized to one limb, to an arm and leg, or to the face, characteristic of stroke localized to the cerebral hemisphere. All patients had magnetic resonance imaging showing infarction of the medial or lateral pontine tegmentum and a patent basilar artery. No definite source for cardiogenic thromboembolism was found. Infarcts in the midline extending from the base of the pons posteriorly into the tegmentum suggested basilar branch occlusion, while infarcts involving only part of the tegmentum probably resulted from small penetrator branch occlusion. Vertigo, light-headedness, or cranial nerve dysfunction suggested a pontine location of neurological dysfunction in these patients, but the nature of the sensory findings did not always predict the lateral, medial, inferior, or superior extent of tegmental infarction.     

The effects of brainstem lesions on vocalization in the squirrel monkey

The present study is an attempt to find out the brain areas involved in the motor coordination of species-specific vocalization. For this purpose, high-frequency coagulations were placed in a systematic manner throughout the brainstem and posterior diencephalon in altogether 43 squirrel monkeys (Saimiri sciureus). The effect of these lesions on different call types elicited by electrical brain stimulation was studied spectrographically. It was found that bilateral destruction of the ventrolateral, ventroposterior and intralaminar thalamus, periventricular and rostral periaqueductal gray, ventral tegmental area of Tsai, nucl. interpeduncularis, nucl. ruber, anterodorsolateral midbrain tegmentum, superior and inferior colliculi, pontine gray, cerebral peduncles, medial pontine reticular formation, raphe and vestibular nuclei did not affect the acoustic structure of the calls tested. On the other hand, lesions in the ventrolateral midbrain involving the substantia nigra and overlying reticular formation, in the midbrain tegmentum just below the inferior colliculus, in the lateral pons and almost the whole medulla (minimal lesion size: 2.5 mm3) changed vocalization significantly. It is suggested that the latter areas are more or less directly involved in the motor coordination of vocalization, while the first are not.     

Pontine control of the urinary bladder and external urethral sphincter in the rat

Neurons in the rostral pontine tegmentum are known to have an important role in controlling micturition. The present experiments used urethane anesthetized rats to examine the effects of electrical stimulation at various sites in the pons on bladder and external urethral sphincter activity and on the volume threshold for inducing micturition. Stimulation with short trains of pulses (50 Hz, 1-3 s trains, 1-15 V) in the laterodorsal tegmental nucleus (LDT), the periaqueductal grey (PAG) or the lateral parabrachial nucleus (L-PBN) elicited contractions of a partially filled, quiescent bladder. However stimulation during a bladder contraction aborted the contraction indicating that these areas have inhibitory as well as excitatory effects. Continuous stimulation (50 Hz) in the PAG or L-PBN during a cystometrogram decreased bladder capacity (mean decrease 36%). Conversely, continuous stimulation in the pontine reticular formation (in or near the dorsal subcoeruleus nucleus and medial parabrachial nucleus) increased bladder capacity (mean increase 50%). Stimulation at pontine sites (LDT, PAG and L-PBN) which elicited bladder contractions also elicited an increase in external urethral sphincter activity. A similar increase in urethral sphincter activity occurred during reflex micturition induced by bladder distension. These data suggest that bladder capacity and the coordination of bladder and external urethral functions are controlled by various neuronal populations in the rostral pons of the rat.     

Spinocerebellar ataxias types 2 and 3: degeneration of the precerebellar nuclei isolates the three phylogenetically defined regions of the cerebellum

Summary. The precerebellar nuclei act as a gate for the entire neocortical, brainstem and spinal cord afferent input destined for the cerebellum. Since no pathoanatomical studies of these nuclei had yet been performed in spinocerebellar ataxia type 2 (SCA2) or type 3 (SCA3), we carried out a detailed postmortem study of the precerebellar nuclei in six SCA2 and seven SCA3 patients in order to further characterize the extent of brainstem degeneration in these ataxic disorders. By means of unconventionally thick serial sections through the brainstem stained for lipofuscin pigment and Nissl material, we could show that all of the precerebellar nuclei (red, pontine, arcuate, prepositus hypoglossal, superior vestibular, lateral vestibular, medial vestibular, interstitial vestibular, spinal vestibular, vermiform, lateral reticular, external cuneate, subventricular, paramedian reticular, intercalate, interfascicular hypoglossal, and conterminal nuclei, pontobulbar body, reticulotegmental nucleus of the pons, inferior olive, and nucleus of Roller) are among the targets of both of the degenerative processes underlying SCA2 and SCA3. These novel findings are in contrast to the current neuropathological literature, which assumes that only a subset of precerebellar nuclei in SCA2 and SCA3 may undergo neurodegeneration. Widespread damage to the precerebellar nuclei separates all three phylogenetically and functionally defined regions of the cerebellum, impairs their physiological functions and thus explains the occurrence of gait, stance, limb and truncal ataxia, dysarthria, truncal and postural instability with disequilibrium, impairments of the vestibulo-ocular reaction and optokinetic nystagmus, slowed and saccadic smooth pursuits, dysmetrical horizontal saccades, and gaze-evoked nystagmus during SCA2 and SCA3.     

Heart rate and blood pressure responses to electrical stimulation of the central nervous system in the pigeon (Columba livia)

An extensive stereotaxic stimulation study of the pigeon brain was conducted with monitoring of heart rate, arterial blood pressure and respiration. Rostrally, short latency tachycardia, hypertension and hyperpnea were elicited from the archistriatum, occipitomesencephalic tract and hypothalamus. In addition, blood pressure decreases followed by long latency tachycardia were elicited from the septal complex, although occasionally slight bradycardia occurred. Tachycardia, hypertension and hyperpnea were elicited from many midbrain sites including the lateral reticular formation, ventrolateral tegmentum, ventral area of Tsai, the midline region between nucleus interpeduncularis and the oculomotor complex, and nucleus mesencephalicus lateralis, pars dorsalis. In addition, moderate tachycardia and hypotension were elicited from the central gray and nucleus intercollicularis while tachycardia, hypertension and hyperpnea were elicited from the tegmental area in the region of the occipitomesencephalic tract. At pontine levels, hypertension and cardioacceleration were elicited from a sparsely celled region lateral to the nucleus abducens and from a ventrolateral tegmental region. With respect to respiratory responses, hyperpnea was elicited from the ventrolateral brainstem at all pontine levels and from the dorsomedial region at rostral pontine levels. In caudal pons apnea was the consistent respiratory response to stimulation of the dorsomedial brainstem. In addition, cardio-acceleration, hypertension and apnea were elicited from the region of the deep cerebellar nucleus cerebellus internus and from its major outflow, the uncinate fasciculus. Finally, stimulation in the medulla elicited bradycardia and hypotension from the vagal rootlets, solitary complex, descending vestibular nucleus and lateral aspect of the dorsal motor nucleus just rostral to the obex. Tachycardia and hypertension were elicited from the medial aspect of the dorsal motor nucleus, medullary reticular formation ventral to the vagal rootlets and ventrolateral medulla.     

A case of hemi-hyperhidrosis and non-paralytic pontine exotropia due to brainstem infarction]

A case of hemihyperhidrosis and non-paralytic pontine exotropia due to brainstem infarction is reported. A 55-year-old hypertensive man developed right hemiparesis with slight dysarthria and nausea upon awaking. The right side of his face and right upper limb and trunk to the level of the Th8-9 territory showed hyperhidrosis, which disappeared in a week. Ocular motor examination revealed that during forward gaze with the left eye fixing, the right eye deviated outward. The patient was able to adduct the right eye to midposition with the right eye fixing. Rightward gaze elicited full abduction and right-beating nystagmus of the right eye, but the left eye did not adduct. When he attempted to gaze leftward, both eyes made the full excursion, but saccades were slow in that direction. Convergence was intact. Vertical gaze was full, and he did not show Horner's sign. This ocular sign, non-paralytic pontine exotropia, disappeared three days later. T2-weighted spin echo magnetic resonance imaging disclosed a small lesion with high intensity in the inner side of the left middle pons. This hyperhidrosis was thought to be caused by destruction of inhibitory fibers thermoregulating sweating. These findings suggest that at the level of the middle pons inhibitory fibers descend along the inner side of facilitatory fibers thermoregulating sweating, which are speculated to descend the dorso-lateral part of the pontine tegmentum. These findings also suggest that lesions of non-paralytic pontine exotropia may be located in the paramedian pontine reticular formation rostral to the abducens nucleus with ipsilateral medial longitudinal fasciculus lesion, but further investigation is necessary.     

Clinical Study on 3 Patients with Infarction of the Vermis/Tonsil in the Cerebellum

Background

Infarction of the vermis and the tonsil in the cerebellum presents as truncal and gait ataxia. Acute rotatory vertigo is often present in infarction of the nodulus in the caudal vermis, which is closely associated with the vestibular pathway, but is minor in infarction of the rostral vermis. The rostral vermis receives input from the dorsal spinocerebellar tract (DSCT) which conveys unconsciousness proprioceptive signals from the ipsilateral lower trunk and leg. The present study investigated the characteristics of infarction of the vermis and the tonsil.

Clinical manifestation of focal cerebellar disease as related to the organization of neural pathways

Neural pathways connect different parts of the cerebellum to different parts of the central nervous system. The cerebellum may be divided anatomically and functionally into three major regions. The cerebellar hemispheres and a small part of the posterior lobe vermis form the pontocerebellum, which receives inputs from the cerebral cortex via the pontine nuclei. The anterior lobe and most of the posterior lobe vermis make up the spinocerebellum, which receives afferents from the spinal cord. The nodulus and flocculus are connected with the vestibular nuclei and constitute the vestibulocerebellum. Most cases of cerebellar disease affect more than one region and different pathways. Hence, they cause generalized cerebellar symptoms dominated by impaired motor control and balance. Focal syndromes after restricted cerebellar lesions are rare. Isolated spinocerebellar affection may give gait ataxia. Vestibulocerebellar disease causes equilibrium disturbances with truncal ataxia and nystagmus. Pontocerebellar lesions typically give ipsilateral limb ataxia, but also dysartria and oculomotor dysfunction if vermal parts are involved. The clinical picture is in most cases of cerebellar disease dominated by motor disturbances, but the cerebellum also participates in the modulation of autonomic and affective responses and in cognitive functions. The cerebrocerebellar and hypothalamocerebellar circuits may be important for these tasks.     

REM sleep behavior disorder in a patient with pontine stroke

Rapid eye movement (REM) sleep behavior disorder (RBD) is frequently seen in patients with neurodegenerative diseases. It can also be seen in brainstem lesions involving the pons. However, RBD in patients with a pure pontine infarction has been rarely reported. A 68-year-old man had a two-month history of violent behavior during sleep. His nocturnal behavior was screaming, thrashing of arms, punching or kicking his spouse, and falling out of bed associated with nightmares of being attacked by what he described as, "terrible animals frightening me." No remarkable neurological signs were found. Brain MRI was performed on this patient and it revealed a lacunar ischemic infarct in the right paramedian pons. On the nocturnal polysomnography (PSG), there was phasic submental and limb electromyographic (EMG) tone and an absence of electroencephalographic (EEG) epileptiform activity during REM sleep. Hence, this patient was diagnosed with pontine infarction and RBD. Treatment with clonazepam 0.25mg nightly resulted in the disappearance of nocturnal events. This case with right pontine tegmental ischemic lesion provides evidence that the unilateral pontine lesion by itself is sufficient to cause RBD.     

Post-infectious Opsoclonus and Reversible Magnetic Resonance Imaging Changes: A Case Report and Review of the Literatures

Purpose: Opsoclonus is a rare neurological disorder in adult. The etiology of opsoclonus includes parainfectious, paraneoplastic, toxic, and metabolic disorders. We reported an old female with post-infectious opsoclonus who had a benign clinical course and reversible brain MRI lesions, and its review of the literature. Case Report: A 67-year-old woman presented with opsoclonus and truncal ataxia for two weeks. The magnetic resonance imaging (MRI) showed the hyperintensity lesions in bilateral medial thalamus, hypothalamus, and tegmentum of pons on Fluid-attenuated inversion recovery (FLAIR) imaging. Investigations of neoplasm and autoimmune disorders showed negative findings. Clinical symptoms subsided in two-week duration and MRI abnormalities also disappeared one month later. Conclusion: A benign clinical course and reversible MRI lesions could be found in the patients with postinfectious opsoclonus such as our case. However, detailed investigations and long-term follow-up are needed to exclude paraneoplastic or other systemic and immunological disorders.     

Depletion of corticotrophin-releasing factor neurons in the pontine micturition area in multiple system atrophy.

We sought to determine whether the putative pontine micturition center in the human dorsal pons contains corticotrophin-releasing factor (CRF) neurons, and whether these neurons are depleted in patients with multiple system atrophy and bladder dysfunction. Brains were obtained at autopsy from 4 control subjects and 4 patients with clinical diagnosis of multiple system atrophy, confirmed neuropathologically. Serial 50 microm cryostat sections were obtained throughout the rostral half of the pons, and every eighth section was processed for CRF immunocytochemistry (rabbit polyclonal antibody). Consecutive sections were stained for nicotinamide adenine dinucleotide phosphate diaphorase (NADPH-d) to identify neurons of the laterodorsal tegmental nucleus or for both CRF and NADPH. Locus ceruleus neurons were identified by their neuromelanin content. Abundant CRF immunoreactive neurons were identified in the dorsal pontine tegmentum just ventral to the locus ceruleus. CRF neurons were intermingled with, but distinct from, the NADPH-d-reactive neurons of the laterodorsal tegmental nucleus. In all multiple system atrophy cases, there was a severe depletion of these CRF-immunoreactive neurons (26.6 +/- 3 neurons/section in patients; 73.7 +/- 4 neurons/section in controls). Our results suggest that depletion of CRF neurons in the putative pontine micturition center may contribute to the severe bladder dysfunction that characterizes multiple system atrophy.     

The clinical and topographic spectrum of cerebellar infarcts: A clinical—magnetic resonance imaging correlation study

We studied 34 consecutive patients with non–mass-producing cerebellar infarcts using a standard protocol of investigations including magnetic resonance imaging (MRI). We analyzed the topography of infarcts to determine the involved arterial territories and we correlated the findings with neurological dysfunction and potential causes of stroke. Sixteen patients had an infarct in the territory of the posterior inferior cerebellar artery (PICA); 2, in the territory of the anterior inferior cerebellar artery (AICA); 13, in the territory of the superior cerebellar artery (SCA); and 8 had junctional infarcts between the territories of the medial and lateral branches of the PICA or PICA/SCA territories. PICA or medial PICA territory infarcts were manifested by acute vertigo and truncal ataxia, while the patients with lateral PICA territory infarcts presented with unsteadiness, limb ataxia and dysmetria without dysarthria. Patients with infarcts in the AICA territory were characterized by limb and trunk ataxia associated with signs of lateropontine involvement. Patients with SCA territory infarcts presented with dysarthria, unsteadiness and/or vertigo, limb ataxia, and dysmetria. Cardiac embolism was the main cause of large infarcts in the territories of the PICA (8/16) or SCA (4/7). Multiple small infarcts were associated with vertebrobasilar atherosclerosis (8/12). These clinical–MRI correlations allow better definition of the topographic and etiological spectrum of cerebellar infarction, which was previously based on pathological studies in subjects with severe infarction.     

Flattened facial colliculus on magnetic resonance imaging in Machado-Joseph disease

Atrophy of the pontine tegmentum and facial colliculus is a characteristic pathological feature of Machado-Joseph disease. We assessed whether this finding can be detected by conventional brain magnetic resonance imaging. A total of 17 patients with genetically confirmed Machado-Joseph disease, 15 disease controls (spinocerebellar ataxia type 6 and dentatorubral-pallidoluysian atrophy), and 17 normal subjects were examined using a 1.5-Tesla magnetic resonance imaging scanner. The widths of the facial colliculus, pontine tegmentum, and pontine base and the area of the fourth ventricle were measured on axial T2-weighted imaging. Pathological examination was performed in 9 Machado-Joseph disease patients. In addition, visual inspection of the facial colliculus was evaluated by receiver operating characteristic analysis. The width of the facial colliculus was significantly smaller in Machado-Joseph disease patients (0.37 ± 0.16 mm; mean ± standard deviation) than in normal subjects (0.73 ± 0.30 mm; P < .01), whereas the width of the pontine tegmentum was smaller in both Machado-Joseph disease (4.85 ± 0.58 mm) and dentatorubral-pallidoluysian atrophy (4.72 ± 0.59) patients than in normal subjects (6.35 ± 0.74 mm; P < .01). Visual evaluation of the facial colliculus showed sufficient area under the receiver operating characteristic curves to differentiate Machado-Joseph disease from dentatorubral-pallidoluysian atrophy (0.78) and spinocerebellar ataxia type 6 (0.87). Pathological evaluation showed significant atrophy of the facial colliculus in all Machado-Joseph disease patients. Atrophy of the facial colliculus is a feasible magnetic resonance imaging finding for diagnosing Machado-Joseph disease, and it is easily found as a flattening of the fourth ventricular floor.     

Joseph Disease with Severe Atrophy of the Brain Stem Tegmentum: Report of an Autopsied Case

An autopsied case of Joseph disease with severe atrophy of the brain stem tegmentum is reported. A Japanese male noticed unsteady gait at age 30 and showed cerebellar ataxia, pyramidal signs, progressive external ophthalmoplegia, peripheral amyotrophy, sensory disturbance, and bradykinesia. He also developed mild dementia and nocturnal delirium at the terminal stage of his illness. His mother, older and younger brothers and daughter also presented progressive ataxia. The mode of inheritance is considered to be autosomal deminant. He died of cardiac failure at age 57. The autopsy revealed significant atrophy of the brain stem and spinal cord. Microscopic examination showed severe degeneration of the dentato-rubral and subthalamo-pallidal systems, pontine nuclei, spinal anterior horn, Clarke's column, accessory cuneate nucleus and spinocerebellar tract, moderate degeneration of the brain stem motor nuclei including the oculomotor nuclei and substantia nigra, and mild degeneration of the spinal posterior and intermediolateral column, and cerebellar cortex. An additional finding, which has received little attention in this disease, was severe atrophy of the brain stem tegmentum associated with degeneration of the reticular formation, raphe nuclei and locus ceruleus. The significance of brain stem tegmental atrophy in Joseph disease is discussed.     

Efferent projections from the mammillary complex of the guinea pig: an autoradiographic study

The efferent projections from the medial and lateral mammillary nuclei of the guinea pig were traced after injecting tritiated amino acid. The major efferent started as the principal mammillary tract, but soon divided into mammillothalamic and mammillotegmental tracts. The mammillothalamic tract projected anterodorsally and terminated in the anterior dorsal, anterior ventral and anterior medial thalamic nuclei. The mammillotegmental tract projected caudally and terminated in the dorsal tegmental nucleus and central gray. The mammillary efferents in the mammillary peduncle ran via the tegmentum of the midbrain and pons. It terminated in the dorsal and ventral tegmental nuclei, basal pontine nucleus and pontine tegmental reticular nucleus. A diffuse mammillary projection had fibers directed dorsally which distributed in the midline thalamic nuclei and in central gray. Rostral projections via the medial forebrain bundle from the medial mammillary nucleus were found in the septal area and diagonal band of Broca. The lateral mammillary nucleus sent fibers which also joined the mammillothalamic and mammillotegmental tracts. These terminated bilaterally mainly in the anterior dorsal and anterior ventral nuclei of the thalamus, and caudally in the dorsal and ventral tegmental nuclei and basal pontine nucleus.     

The dysarthria-clumsy hand syndrome: a distinct clinical entity related to pontine infarction

Using magnetic resonance imaging, we studied 6 patients with the dysarthria-clumsy hand syndrome. All were found to have pontine infarctions contralateral to the symptomatic side. Clinically, these patients exhibited dysarthria; "clumsiness," characterized by dysmetria, dysrhythmia, dysdiadochokinesia and sometimes truncal and gait ataxia; and mild ipsilateral weakness. Previous clinical-anatomical correlations for this syndrome are limited by inconsistencies in clinical diagnostic criteria and low-resolution imaging methods. In our patients, and in a review of the literature, the overwhelming majority of patients with the dysarthria-clumsy hand syndrome had pontine infarcts. We conclude that if rigid clinical criteria are used, the label of the dysarthria-clumsy hand syndrome predicts a lesion in the contralateral basis pontis.     

Tectal projections in the goldfish (Carassius auratus): a degeneration study.

Efferents revealed by degeneration staining following tectal lesions in goldfish are presented. Four major projections were found. Ascending ipsilateral projections to pretectal-diencephalic areas exit the tectum rostrally and laterally and terminate in the area pretectalis (AP), lateral geniculate (LGN), nucleus pretectalis (NP), and nucleus rotundus (NR). Ascending contralateral projections exit rostrally and possibly laterally, enter the posterior and postoptic commissures and terminate in the contralateral AP, LGN, NP, NR, and rostral tectum. A medially directed projection enters the intertectal commissure, and some of these fibers may terminate sparsely in an area of the contralateral tectum homotopic to the lesion. A descending projection exits the tectum laterally and projects ipsilaterally to a dorsolateral tegmental nucleus (DLT) and the lateral reticular formation of the tegmentum and pons, and contralaterally to the medial reticular formation of the tegmentum and pons.     

Role of the periaqueductal grey in vocal expression of emotion

In 32 squirrel monkeys (Saimiri sciureus) the role of the periaqueductal grey has been investigated by combined stimulation/lesioning and by neuroanatomical experiments. The results are as follows. Firstly, periaqueductal lesions invading the laterally adjacent tegmentum abolish species-specific calls elicitable by electrical brain stimulation. This holds for stimulation sites rostral as well as caudal to this area. The only vocalizations which survive are phonations of an artificial character which can be evoked from the lateral medulla. Spontaneous vocalizations also seem to be abolished. Secondly, vocalizations elicited from the periaqueductal grey are not affected by bilateral lesions in vocalization-eliciting areas rostral to it, but are abolished by lesions in the dorsolateral pons and ventrolateral medulla. Thirdly, the periaqueductal grey receives direct projections from all vocalization-eliciting areas tested, viz. the precallosal cingulate gyrus, gyrus rectus, medial amygdata, central amygdaloid nucleus/substantia innominata, nucleus striae terminalis, dorsal hypothalamus, midline thalamus, periventricular grey, dorsolateral and ventrolateral midbrain tegmentum. Fourthly, the periaqueductal grey projects directly to the nucleus ambiguus, the site of the laryngeal motoneurones. The course of the main bulk of fibres corresponds to the lesion sites effective in abolishing periaqueductally elicited vocalizations. From these results, it was concluded that the caudal periaqueductal-lateral tegmental area is a necessary relay station for all external and internal stimuli capable of inducing species-specific calls. Its position within the stimulus-response loop seems to be on the output side, immediately above the level of motor-corrdination but below that of stimulus recognition.     

Familial spinocerebellar degeneration as an expression of adrenoleukodystrophy.

A family with adrenoleukodystrophy and clinical manifestations of spinocerebellar degeneration was studied. Two adult male first cousins showed progressive limb and truncal ataxia, slurred speech and spasticity of the extremities. Brain CT scans demonstrated atrophy of the pons and cerebellum, in both cases. Very long chain fatty acids in plasma and erythrocyte membranes were elevated in the affected patients and intermediately increased in an aunt and the mother of one patient, thereby indicating homozygotes and carriers of adrenoleukodystrophy, respectively. This unusual type of adrenoleukodystrophy seems to be transmitted as an X-linked recessive trait.     

A case showing Wallerian degeneration of the bilateral middle cerebellar peduncles on MRI followed by the right pontine infarction]

We report a 63-year-old man who presented with the left facial palsy, the left hemiparesis, the left limb ataxia, and the bilateral truncal ataxia. On admission, magnetic resonance imaging (MRI) showed an abnormal high intensity lesion at the right paramedian region of the upper to middle pons on T2-weighted images (T2WI). He was diagnosed as having a pontine lacunar infarction. The contralateral cerebellar lesions were caused by involvement of the pontocerebellar fibers. On the 29th day from the onset, MRI showed the new abnormal high intensity lesions at the bilateral middle cerebellar peduncles on T2WI. These lesions were supposed to be Wallerian degeneration caused by involvement of the pontocerebellar fibers. This case suggests that Wallerian degeneration occurs followed by a unilateral infarction involving pontocerebellar fibers.