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PubMedDate not supplied

Neuropathology of central respiratory dysfunction in infancy.

We studied the neuropathology of 7 infants who had primary respiratory problems unrelated to increased intracranial pressure. These infants ranged in age from newborn to 2 years. Five were male. In 2 of them the main neuropathological findings were in the brainstem with prominent neuroglial heterotopia in the subarachnoid space, and aplasia of the VI and VII cranial nerves. Two infants had abnormalities of the X and XII nerves together with neuronal heterotopia and migration failure of the inferior olivary nuclei. In 1 infant diagnosed with Ondine's curse, examination showed diffuse neuronal loss and gliosis in the medullary tegmentum. One infant had a unilateral infarction in the medulla and another showed extensive gliosis in the brainstem tegmentum along with a large infarction in the region of the anterior cerebral artery. These infants exhibited a spectrum of abnormalities including neuronal dysplasia, gliosis and hypoxic-ischemic changes. In the differential diagnosis of respiratory dysfunction in infants a rare consideration is a central etiology based on malformation of essential neuronal components of the brainstem.

Agenesis of Corpus Callosum↗

PubMed1994

Prediction of outcome in children with congenital hemiplegia: a magnetic resonance imaging study.

The degree of Wallerian degeneration (WD) in the corticospinal tracts seen with magnetic resonance imaging (MRI) was correlated with the distribution and severity of congenital hemiplegia in 20 children aged nine months to nine years. All the children had hemispheric lesions diagnosed with ultrasound in the neonatal period: MRI and clinical assessment were performed from nine months to nine years of age. Hemiplegia was graded as mild, moderate or severe and into predominantly upper or lower limb distribution. WD was assessed by the presence or absence of signal intensity changes in the internal capsule on inversion recovery and spin echo sequences and by the asymmetry of the upper brainstem. The degree of asymmetry was estimated by measuring the cross sectional area (CSA) of the brainstem at three levels and calculating the ratio of the measurements between the side of the lesion and the unaffected side. Infarct size was estimated from the CSA of the infarct at the maximum site of the lesion. Both measurements were correlated with the severity of outcome and the site of involvement. There was a better correlation between severity of outcome and brainstem asymmetry (p = 0.003) than severity of outcome and infarct size (p = 0.02). There was also a significant correlation between upper limb involvement and brainstem asymmetry (p = 0.01). As WD estimated by brainstem asymmetry appears early and is easy to measure, it may be a good marker to estimate later impairment in infants with predominantly unilateral hemispheric haemorrhagic/ischaemic lesions diagnosed in the neonatal period.

Brain Stem↗

PubMed1999

Clinical application of three-dimensional anisotropy contrast magnetic resonance axonography. Technical note.

The utility of three-dimensional anisotropy contrast (3DAC) magnetic resonance (MR) axonography, a method sensitive to neuronal fibers and their directionality, was investigated in the clinical setting using a 3-tesla MR imaging system based on a General Electric Signa platform. The study focused on healthy volunteers and patients with common structural central nervous system disorders, namely chronic infarction, brainstem cavernous hemangioma, supratentorial meningioma, and astrocytoma. Three orthogonal anisotropic diffusion-weighted images were first obtained. Three primary colors were each assigned to a diffusion-weighted image, respectively, and the images were subsequently combined into a single-color image in full-color spectrum (3DAC MR axonography image). Fiber-tract definition in the cerebral peduncle of the midbrain of healthy volunteers showed intersubject variation, with two general patterns recognized: dispersed (60% of cases) and compact (40% of cases). Pathological alterations in the fiber tracts were readily identified in cases involving wallerian degeneration of the pyramidal tract, as illustrated in the cases of chronic infarction. Displacement of major tracts, such as the medial lemniscus or corticospinal tract, as well as fiber directionality, was also easily recognized in cases of mass lesions. As an imaging method uniquely capable of providing information regarding axonal connectivity, 3DAC MR axonography appears to have promising potential for routine clinical application.

Astrocytoma↗

PubMed1990

Respiratory failure and unilateral caudal brainstem infarction.

We report clinicotopographic correlations in 2 patients with central hypoventilation and unilateral infarct in the caudal brainstem. One patient had nearly complete loss of ventilation involving both automatic and voluntary components. He showed no ventilator response during a CO2 retention test (PaCO2 62 mm Hg, PaO2 82 mm Hg), while consciousness was preserved until death. The infarct involved the reticular formation, nucleus tractus solitarius, nucleus ambiguus, and nucleus retroambiguus on the right but spared the dorsal motor nucleus of the tenth cranial nerve, and sensory and corticospinal tracts. The second patient showed hypoventilation more selectively involving automatic responses (Ondine's curse). The infarct involved the medullary reticular formation and nucleus ambiguus but spared the nucleus tractus solitarius. We suggest that unilateral involvement of pontomedullary reticular formation and nucleus ambiguus is sufficient for generating loss of automatic respiration, while associated lesion of the nucleus tractus solitarius may lead to more severe respiratory failure involving both automatic and voluntary responses.

Aged↗

PubMed1987

See-saw nystagmus and brainstem infarction: MRI findings.

A patient with see-saw nystagmus had a lesion localized by Magnetic Resonance Imaging (MRI) to the paramedian ventral midbrain with involvement of the right interstitial nucleus of Cajal. This the first MRI study of see-saw nystagmus associated with a presumed brainstem vascular event. Our findings support animal and human studies suggesting that dysfunction of the interstitial nucleus of Cajal or its connections is central in this disorder.

Brain Stem↗

PubMed1987

Brainstem death with persistent EEG activity: evaluation by xenon-enhanced computed tomography.

A patient with brainstem infarction met the clinical criteria for brain death but had persistent EEG activity, complicating our decision to withdraw life support. We evaluated cerebral blood flow with xenon-enhanced computed tomography (Xe/CT), which documented the absence of posterior circulation flow and persistent, low, anterior circulation flow. This information led us to withdraw life support, despite the presence of EEG activity. The Xe/CT method noninvasively measures local cerebral blood flow and may enhance diagnostic certainty in complicated brain-death evaluations.

Brain Death↗

PubMed1986

Clinical and electrophysiological study in a patient surviving from locked-in syndrome.

The results of clinical and electrophysiological investigations in a patient with a locked-in syndrome due to a pontine infarction, mainly on the right side, are presented. EEG showed only slight disturbances, while BAER and SSER revealed response alterations as could be expected from physical examination. BAER revealed altered central conduction after stimulation of either side. After median nerve stimulation on the right side SSER was slightly delayed, while no reproducible cortical response was seen after stimulation on the left side. Our patient survived and showed partial recovery.

Audiometry, Pure-Tone↗

PubMed1988

[Magnetic resonance imaging in horizontal oculomotor paralysis caused by infarction].

Four patients with focal brainstem ischemic strokes and various types of horizontal oculomotor disturbances have been studied clinically and radiologically. One had a six nerve palsy, one a unilateral internuclear ophthalmoplegia, one a Fisher's one-and-a-half syndrome, and one a paramedian pontine reticular formation syndrome with a sixth nerve palsy. In all patients a C.T. Scan and a Magnetic Resonance Imaging (MRI) were obtained. The MRI study was performed on a CGR Magniscan 5000 with a superconducting magnet of 0.5 Tesla. In all patients a hypersignal in T2 weighted images was shown and corresponded to the brainstem infarct. MRI allowed accurate delineation of the lesion and clinico-radiologic correlations in three patients. But the low specificity of MRI does not permit to distinguish edema from necrosis, gliosis or demyelination in a region with a pathological MRI signal. In one patient this low specificity and perhaps partial volume effects decreased the accuracy of the clinico-topographic correlation.

Abducens Nerve↗

PubMed1992

Treatment of cerebellar infarction by decompressive suboccipital craniectomy.

BACKGROUND AND PURPOSE: We present an anecdotal series of 11 patients without past history of stroke with progressive neurological deterioration while on medical therapy for large cerebellar infarctions. Clinical signs of brain stem compression developed in these patients. Computerized tomography of the head confirmed mass effect from brain edema. It was the clinical judgment of the neurologists and neurosurgeons that each of these 11 patients would expire without surgical intervention. METHODS: All 11 patients (seven men, four women; mean age, 54 years) were treated with suboccipital craniectomy for decompression and temporary ventriculostomy for cerebrospinal fluid pressure monitoring and drainage. RESULTS: Seven patients demonstrated neurological improvement on the first postoperative day. Two patients returned to their previous jobs 3 months after surgery. The Barthel Index indicated that six individuals were functioning with minimal assistance within a follow-up period of 16-60 months. The remaining three were functionally dependent. No mortality was noted in this series. CONCLUSIONS: These results suggest that decompressive suboccipital craniectomy may be an effective, lifesaving procedure for malignant cerebellar edema after a large infarction.

Adult↗

PubMed1984

Neck manipulation causing stroke. Case reports.

Neck manipulation may cause injury to the vertebrobasilar arterial system with resultant brainstem ischaemia or infarction. This complication has been recognized for many years, and although rarely reported in the literature is a well-recognized problem encountered by neurologists. This article describes 3 patients with this condition seen at the Johannesburg Hospital. The probable pathogenesis is that a thrombus occurs at a site of vertebral artery injury created by sudden sharp neck movement chiefly between the axis and atlas. This thrombus may propagate or embolize. The therapeutic role of anticoagulants is discussed.

Adult↗

PubMed1991

[A case of pregnant woman with lupus anticoagulant positive systemic lupus erythematosus complicated with intrauterine fetal death, eclampsia, and brainstem vascular damage].

A 28-year-old female patient with SLE was found to have complications from intrauterine fetal death, eclampsia, and brainstem vascular damage during pregnancy. These symptoms were associated with lupus anticoagulant (LAC). An LAC positive SLE case with accompanying cerebrovascular disease and fetal loss at the same time has rarely been reported. The first brain CT scan revealed the presence of multiple middle pons infarction. Ten days after onset, a brain MRI showed the change into hemorrhagic infarction in the same region, suggesting disseminated intravascular coagulation (DIC) caused by severe obstetric complications. Although the severity of the brain damage implied poor prognosis, her symptoms were alleviated by intensive care with a large amount of steroid therapy and active rehabilitation. LAC-positive SLE patients are apt to encounter obstetric complications in pregnancy. Therefore they must be treated by medical specialists in cooperation with gynecologists. When they also have accompanying with cerebrovascular diseases, active therapy for SLE is necessary for a good prognosis.

Adult↗

PubMed1981

Abnormal brainstem auditory evoked potentials and neuropathology in "locked-in" syndrome.

Brainstem auditory evoked potentials (BAEPs) were studied in a "locked-in" syndrome resulting from multiple occlusions of paramedian pontine arteries. Abnormality of BAEPs (first recorded 10 days after infarction) indicated that brainstem damage extended beyond the basis pontis, where, typically, interruption of corticospinal pathways results in the "locked-in" syndrome. Later, partial BAEP recovery suggested that the technique can be used to distinguish between permanent and transient brainstem involvement. Autopsy examination revealed close correspondence between permanent brainstem damage and persistent BAEP abnormalities. Persistent abnormal prolongation of III-V conduction time and aberration of wave IV were associated with damage near the lateral lemniscus contralateral to the stimulated ear.

Aged↗

PubMedDate not supplied

Morse-code-controlled computer aid for the nonvocal quadriplegic.

A computer-based communications system was established for a quadriplegic patient who suffered a brainstem infarction that left him with normal intelligence, but without speech and without functional motor control except for the thumb of his right hand. An arm brace was developed to support the hand and thumb so it could control two switches that generated Morse code signals. An interface was also devised to translate Morse code into ASCII characters to access a personal computer. Additional codes were added to represent keyboard control functions. This device allows the patient to communicate with others and control, to some extent, his environment.

Brain Stem↗

PubMed1991

Word deafness and auditory cortical function. A case history and hypothesis.

A patient who already had Wernicke's aphasia due to a left temporal lobe lesion suffered a severe deterioration specifically of auditory language comprehension, subsequent to right temporal lobe infarction. A detailed comparison of his new condition with his language status before the second stroke revealed that the newly acquired deficit was limited to tasks related to auditory input. Further investigations demonstrated a speech perceptual disorder, which we analysed as due to deficits both at the level of general auditory processes and at the level of phonetic analysis. We discuss some arguments related to hemisphere specialization of phonetic processing and to the disconnection explanation of word deafness that support the hypothesis of word deafness being generally caused by mixed deficits.

Aphasia, Wernicke↗

PubMed1977

Alpha-pattern coma: 24 cases with 9 survivors.

Alpha-pattern coma denotes the association of a comatose state with an electroencephalogram consisting predominately of alpha-frequency activity. Over the past three years we have studied 24 such cases: 14 following cardiopulmonary arrest, 6 following respiratory arrest, and 3 following brainstem infarction. Of the 9 patients who survived, only 1 had a significant neurological deficit. It was found that: (1) alpha-pattern coma can result from a variety of neurological insults; (2) a significant proportion of patients survive, often with little or no deficit; (3) clinical evidence of intact brainstem function implies a favorable outcome; (4) the electroencephalogram was similar in survivors and nonsurvivors and did not differentiate diffuse cerebral dysfunction from focal brainstem disease; and (5) neuropathological studies demonstrated diffuse cortical as well as brainstem changes.

Adult↗

PubMed2006

Magnetic resonance imaging changes in Fabry disease.

UNLABELLED: Recognized magnetic resonance imaging (MRI) abnormalities in the brains of patients with Fabry disease include the consequences of infarction and haemorrhage, non-specific white and grey matter lesions, vascular anomalies, in particular dolicho-ectasia, and a characteristic appearance of the posterior thalamus. A preliminary analysis of MRI findings in patients registered in FOS, the Fabry Outcome Survey, indicates that most patients had abnormal scans (25/47). The commonest abnormality, in males and females, was the presence of cerebral white matter lesions, the number of which increased with patient age. CONCLUSION: MRI is a valuable resource for assessing the CNS complications of Fabry disease, and their response to time and treatment.

Brain↗

PubMed1990

Vertical gaze palsy and selective unilateral infarction of the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF).

We report a clinico-pathological correlation study in a patient with basilar artery thrombosis, who developed tetraplegia and combined up- and downgaze palsy involving voluntary saccades and visually-guided movements, but sparing the oculocephalic responses. At necropsy, apart from bilateral infarction in the basis pontis, there was a single unilateral infarct selectively destroying the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF) on the right. The posterior commissure and its nucleus, the nucleus of Cajal, the nucleus of Darkschewitsch and the pontine tegmentum were spared. We suggest that the unilateral riMLF lesion may have disrupted bilateral upgaze excitatory and inhibitory inputs and unilateral downgaze excitatory inputs. The functional anatomy of inhibitory and excitatory vertical gaze circuitry, which remains speculative, may explain why a unilateral lesion of the upper midbrain tegmentum may be sufficient to generate an upgaze palsy or a combined up- and downgaze palsy, while an isolated downgaze palsy requires bilateral lesions.

Brain Stem↗

PubMed1998

[A case of non-paralytic pontine exotropia due to brainstem infarction].

A case with non-paralytic pontine exotropia due to brainstem infarction is reported. A 58-year-old male developed sudden onset diplopia. Ocular motor findings were as follows; in forward gaze, the left eye was in abduction position. Leftward gaze, the right eye did not adduct with left beating nystagmus of left eye. Rightward gaze, both eyes could make full excursion and upward, downward gaze were possible. These findings was noted at only acute phase, 7 days later NPPE was disappeared. MRI revealed spotty lesion in the paramedian portion of the mid pontine tegmentum. It is important to observe ocular movement at acute phase because NPPE is very important sign in diagnosis of brainstem disorders.

Brain Stem↗