Brainstem Signs Research Articles

Progressive encephalomyelitis with rigidity and myoclonus preceding otherwise asymptomatic Hodgkin's lymphoma

Stiff-person syndrome (SPS), a rare neuroimmunological disorder, is characterized by symmetrical rigidity and muscle stiffness, particularly of axial and proximal limb muscles. Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a variant of SPS which includes additional clinical features (e.g. sensory symptoms, brain stem signs and pathological CSF findings). An association of both SPS and PERM with (solid) malignancies has been previously reported. Beyond this, there have been single reports of SPS in the setting of Hodgkin's lymphoma (HL). Here, we present a case of PERM associated with HL, with PERM preceding occurrence of lymphoma by more than seven months. Our observation has obvious implications for the management and, in particular, diagnostic evaluation of patients with PERM.

Poor-Grade Ruptured Middle Cerebral Artery Aneurysm With Intracerebral Hematoma: Bleeding Characteristics and Management

Poor-grade ruptured middle cerebral artery aneurysm is frequently associated with intraparenchymal hemorrhage, which is associated with high morbidity rates. We analyzed the clinical presentations and surgical strategies of 23 cases of ruptured middle cerebral artery aneurysm. Hematomas were divided into three types: temporal hematoma (7 patients), sylvian hematoma (10 patients), and frontal hematoma (6 patients). In 13 of 23 patients, preoperative brainstem symptoms suggested impeding uncal herniation. Surgical procedures included external decompression in 11 patients, simple lateral temporal lobectomy in 5, and selective uncectomy in 9. Three patients died. Favorable outcome defined as upper half of severely disabled or better in the extended Glasgow Outcome Scale was achieved in 13 patients. Patients with frontal hematomas presented with both uncal herniation and brainstem signs preoperatively, but this subgroup showed unexpectedly good recovery. Patients with sylvian hematomas had relatively poor outcomes. The present series suggests that aggressive decompression and evacuation of hematoma in the acute stage may prevent significant postoperative brain swelling, and will not compromise the treatment of vasospasm.

Degeneración walleriana bilateral de las fibras pontocerebelosas secundaria a ictus pontinos: presentación de 4 casos

Wallerian degeneration occurs after demyelination of the distal neuronal axons due to proximal damage of any type. We present the magnetic resonance findings in four patients with brainstem stroke and signs of Wallerian degeneration in the pontocerebellar tracts. We reviewed the magnetic resonance studies in four patients with subacute or chronic stage pontine lesions and the signal alterations at the level of the medial cerebellar peduncles. We correlated the findings in T2-weighted sequences and diffusion-weighted sequences with the time of evolution and etiology of the stroke.

Delayed cerebral thrombosis after initial good recovery from pneumococcal meningitis

To report an unusual clinical course in 6 patients with community-acquired acute bacterial meningitis and to compare clinical features with cases reported in the literature. Case series from Dutch hospitals from 2003 to 2008. Five out of six patients were male, age ranged from 30 to 73 years (mean age, 47 years). All patients had pneumococcal meningitis, received adjunctive dexamethasone treatment on admission, and made a good or excellent initial recovery. After 7 to 19 days, patients suddenly deteriorated, developing headache, fever, a decreased level of consciousness, brainstem signs, or hemiparesis. Imaging studies showed infarctions involving the thalamus or brainstem in all patients. Repeated lumbar puncture showed a pleocytosis, but CSF cultures were sterile. Five patients were treated with high-dose steroids on deterioration. Outcome was poor: 4 patients died and 2 remained disabled. Autopsies, performed in 2 patients, showed infarctions predominantly involving the posterior circulation territory, thrombosis in penetrating arteries, but no evidence of vasculitis. We identified 5 meningitis cases with delayed vasculopathy in the literature, but these patients did not exactly resemble the clinical course of our patients. Delayed cerebral thrombosis may occur in patients with excellent recovery from pneumococcal meningitis. All patients were treated initially with adjunctive dexamethasone therapy, suggesting a dexamethasone-associated effect. Pathology suggests an immunologic reaction targeting cerebral blood vessels.

Evaluating the Affective Component of the Cerebellar Cognitive Affective Syndrome

Evaluating the Affective Component of the Cerebellar Cognitive Affective Syndrome

Isolated third cranial nerve palsy: a rare presentation of neurocysticercosis

Isolated third cranial nerve palsy is usually due to vascular causes like posterior communicating artery aneurysm and it is rare in neurocysticercosis. Third cranial nerve palsy in neurocysticercosis is usually caused by supratentorial or sub-arachnoid lesions with accompanying hydrocephalus or meningitis. We report a patient who presented with isolated third cranial nerve palsy without any other brainstem signs caused by neurocysticercosis involving the midbrain.

Studies on the natural history of multiple sclerosis. 7. Correlates of clinical change in an early bout.

Factors of predictive value for the course of an early bout of multiple sclerosis were sought from a nationwide series of over 500 men who, before and during the bout under study, were on active duty in the U.S. Army. Most features of the neurologic involvement, as well as spinal fluid and season, were unrelated to change in hospital. Outcome was statistically associated with brain stem signs and age, but in an irregular fashion. Those for whom this was the onset bout (Group B) and those given “specific” treatment changed more for better and worse. The major correlates with improvement were short duration of the bout, sensory signs, and overall severity of neurologic impairment at admission; some inverse relationship was seen with total duration of illness for those with prior bouts (Group A). Bout duration and severity seemed additive in their effects: Of those with short and severe bouts, 95 per cent improved and none worsened; of those with long and mild or moderate bouts, 12 per cent improved and 17 per cent worsened. The relation with bout duration has been found in other series, but not that with severity.

Clinical correlates of abnormal brain-stem auditory evoked responses in multiple sclerosis.

Brain-stem auditory evoked responses (BAERs) were examined in 178 patients with multiple sclerosis (MS) and compared to the frequency of abnormalities in visually evoked responses (VERs) and in CSF electrophoresis. In clinically definite MS, BAERs were abnormal in 61% and a significant relationship was noted between disability due to MS and the frequency and severity of BAER abnormalities. In suspected MS, BAERs showed evidence of a second lesion in 14% whereas VERs indicated a second lesion in 24%. Abnormal BAERs in patients with suspected MS with brain-stem signs were significantly associated with the presence of truncal and limb ataxia. In progressive possible MS, abnormal BAERs were found in 49% but indicated a second lesion in 35% of patients and were significantly related to the duration of illness. In progressive possible MS, abnormal VERs but not abnormal BAERs, were significantly associated with the presence of CSF oligoclonal IgG banding. Normal BAERs in association with clinical brain-stem abnormalities were found in 24% of patients with clinically definite MS, 50% with suspected MS and 33% with progressive possible MS.

Brain death and the apnea test

Brain death and brainstem death are two important medicolegal issues that have drawn much public interest in the recent past. Pronouncement of death has important ethical, social, psychological, and medical dimensions. In this issue of the journal we publish a technical note on the apnea test for the diagnosis of brainstem death. We reiterate that brainstem death is a conclusion drawn on the basis of several findings. An in-depth discussion on brainstem death is beyond the scope of this commentary. The clinician is often called upon to comment on brainstem activity or inactivity in order to pronounce brainstem death. We would like to stress that the clinician should be satisfied with identifying the precise cause of coma, its irreversibility, and the demonstration of irreversible failure of all brainstem functions. The bedside tests for confirming the failure of brainstem functions include the pupillary light reflex (midbrain function); the corneal reflex, the oculocephalic reflex, and the ice-cold caloric test (pontine functions); and the gag reflex, the cough reflex, the atropine test for vagus-mediated tachycardia, and the apnea test (medullary function). These tests need to be carried out by clinicians who are not part of the treating team and organ donation / harvesting team and who have sufficient expertise in the test(s). It is important to demonstrate the irreversibility of brainstem failure by confirming the absence of these brainstem signs after an interval of 6–24 h. The laws and guidelines pertaining to death vary from country to country and between states in some countries.[1] Each country has its own laws and rules regarding brainstem death and the procedure to be followed for confirming it. The tests need to be carried out only after satisfying the prerequisites. The standard procedures need to be carefully adopted. It is important that the family or friends of the subject are taken into confidence and their consent obtained after a detailed discussion. A recent article has drawn attention to the various pitfalls while making a diagnosis of brain death.[1] The purpose of this technical report is to bring to the attention of the readers the intricacies of the apnea test and to familiarize them with the commonly followed techniques. The apnea test is only one of the many tests that are done to ascertain medullary function. It should be interpreted in the light of the other tests for brain death and the underlying disorder and recent medications received by the patient. We recommend that each institution set up its own guidelines (in the light of the existing laws) on when to invoke the brainstem death procedures, how to carry it out, what would be the clinical context in which it would be ordered, who would order the tests, who would carry out the tests, and how it would be documented in the records. It will be worthwhile to have occasional clinical audits of the issue and update the guidelines periodically.

Progression of acute cochleovestibulopathy into anterior inferior cerebellar artery infarction

Antemortem diagnosis of isolated inner ear infarction remains a diagnostic challenge since current imaging techniques do not permit differentiation of isolated labyrinthine infarction from other more benign disorders involving the inner ear. An 81-year-old woman with diabetes, hypertension and hypercholesterolemia presented with vertigo and hearing loss. Initial examination revealed findings of left cochleovestibular labyrinthitis without brainstem or cerebellar signs. MRIs including the diffusion-weighted images were normal. Five days later, however, she developed dysarthria, and decreased facial sensation, subtle facial palsy, and dysmetria in the left side. Follow-up MRI was consistent with acute infarctions in the territory of anterior inferior cerebellar artery (AICA), involving the left lateral pons, left middle cerebellar peduncle, and inferolateral cerebellum. AICA infarction should be considered in acute audiovestibular syndrome, especially in aged patients with vascular risk factors, even though the classic brainstem or cerebellar signs are absent.

Opticospinal multiple sclerosis in Iran

Clinical course, magnetic resonance imaging (MRI) findings and cerebrospinal fluid data (CSF) on 20 patients among 520 with clinically definite multiple sclerosis (MS) according to the criteria of Poser et al., with opticospinal clinical presentation above 5 years and mean disease duration of 8 ± 4.4 years were reviewed. The prevalence rate was 3.8%. The clinical course was relapsing-remitting (RR) for all patients. The mean age of onset was 24 ± 8.2 years. The gender ratio was 2.3:1 female:male. First clinical presentation was spinal signs in 12 (60%), optic neuritis in 7 (35%) and simultaneous involvement of both eyes in 1 (5%) patient. No transverse myelitis (TM), sustained severe optic neuritis (SSON) or minor brain stem signs were recorded. No conversion to conventional MS (CMS) or secondary progressive MS (SPMS) was identified during the study period and no case had positive family history for the disease. The yearly number of attacks was 0.66 ± 0.84, with mean Kurtzke expanded disability status scale (EDSS) of 2.5 ± 1.2. All patients had 1 to 5 hemispheral T2 lesions in brain MRI, non-fulfilled Barkhof criteria for brain lesions. All had preventricular and 14 (70%) had juxtacortical lesions. None had gadolinium T1 enhanced lesions. No involvement of the brainstem or cerebellar structures was detected. Fifteen had 1 cervical lesion, five had 2 and four had 1 concomitant thoracic cord signal, all extending below three vertebral segments in sagittal planes with peripheral white matter location on the axial planes. Spinal fluid contained normal cell and protein with negative oligoclonal bands (OB).

Recurrent audiovestibular disturbance initially mimicking Ménière’s disease in a patient with anterior inferior cerebellar infarction

An anterior inferior cerebellar artery (AICA) stroke is characterized by vertigo, tinnitus, and deafness in addition to facial weakness, hemiataxia, and hypalgesia. Sometimes, it can present as sudden deafness with vertigo, without brainstem or cerebellar signs. We report a 55-year-old woman with hypertension and diabetes, showing recurrent audiovestibular disturbance before a typical pattern of AICA infarction, which was initially diagnosed as Ménière's disease. In elderly patients with recurrent hearing loss and vertigo lasting several minutes, lack classic brainstem or cerebellar signs, if they have vascular risk factors, physicians may also consider the potential symptom of AICA infarction.

Adult-onset Alexander disease: a series of eleven unrelated cases with review of the literature

Alexander disease (AD) in its typical form is an infantile lethal leucodystrophy, characterized pathologically by Rosenthal fibre accumulation. Following the identification of glial fibrillary acidic protein (GFAP) gene as the causative gene, cases of adult-onset AD (AOAD) are being described with increasing frequency. AOAD has a different clinical and neuroradiological presentation with respect to early-onset AD, as abnormalities are mainly concentrated in the brainstem-spinal cord junction. We report detailed clinical and genetic data of 11 cases of AOAD, observed over a 4-year period, and a review of the previously reported 25 cases of genetically confirmed AOAD. In our series, onset occurred as late as age 62, and up to 71 in an affected deceased relative. Most cases appeared sporadic, but family history may be misleading. The most frequent symptoms were related to bulbar dysfunction-with dysarthria, dysphagia, dysphonia (seven patients)-, pyramidal involvement (seven patients) and cerebellar ataxia (seven patients). Four patients had palatal myoclonus. Sleep disorders were also observed (four cases). Bulbar symptoms, however, were infrequent at onset and two symptomatic patients had an almost pure pyramidal involvement. Two subjects were asymptomatic. Misdiagnosis at presentation was frequent and MRI was instrumental in suggesting the correct diagnosis by showing, in all cases, mild to severe atrophy of the medulla oblongata extending caudally to the cervical spinal cord. In ten patients, molecular studies revealed six novel missense mutations and three previously reported changes in GFAP. The last typical patient carried no definitely pathogenic mutation, but a missense variant (p.D157N), supposedly a rare polymorphism. Revision of the literature and the present series indicate that the clinical picture is not specific, but AOAD must be considered in patients of any age with lower brainstem signs. When present, palatal myoclonus is strongly suggestive. Pyramidal involvement, cerebellar ataxia and urinary disturbances are common. Less frequent findings include sleep disorders and dysautonomia. Fluctuations may occur. The course is variable, usually slowly progressive and less severe than the AD forms with earlier onset. AOAD is more common than previously thought and might even be the most common form of AD. The diagnosis is strongly suggested by MRI and confirmed by GFAP gene analysis.

A practical approach to acute vertigo

Patients complaining of symptoms of acute vertigo present a diagnostic challenge for the clinician; the main differential diagnoses are acute unilateral peripheral vestibulopathy (“vestibular neuritis”), cerebellar stroke or migraine. The...

Pharmakotherapie zentraler Nystagmusformen

Nystagmus causes blurred vision due to oscillopsia, as well as impaired balance. Depending on etiology, additional cerebellar and brain stem signs may occur. We present the current pharmacotherapy of the most common forms of central nystagmus: downbeat nystagmus (DBN), upbeat nystagmus (UBN), acquired pendular nystagmus (APN), and congenital nystagmus (CGN). Recommended medical therapies are aminopyridines (4-AP) for DBN and UBN, gabapentin and memantine for CGN and APN, and baclofen for periodic alternating nystagmus (PAN).

Sudden-Onset Bilateral Hearing Loss: Case Report

An ischaemic infarction of the anterior inferior cerebellar artery (AICA) is usually associated with vertigo, hearing loss, facial palsy, nystagmus, or truncal ataxia; it is often accompanied by other brainstem or cerebellar signs. Sudden- onset bilateral hearing loss without associated neurological symptoms is infrequent in the literature. We report a case of sudden bilateral hearing loss, later diagnosed as AICA infarction without other symptoms.

Lumbar Puncture and Brain Herniation in Acute Bacterial Meningitis: A Review

There has been controversy regarding the risk of cerebral herniation caused by a lumbar puncture (LP) in acute bacterial meningitis (ABM). This review discusses in detail the issues involved in this controversy. Cerebral herniation occurs in about 5% of patients with ABM, accounting for about 30% of the mortality. In many reports, LP is temporally strongly associated with this event of herniation and is most likely causative based on pathophysiologic arguments. Although a computed tomography (CT) scan of the head is useful to find contraindications to an LP, a normal CT scan in ABM does not mean that an LP is safe. Clinical signs of "impending" herniation are the best predictors of when to delay an LP because of the risk of precipitating herniation, even with a normal CT scan. Some of these clinical signs to be considered are deteriorating level of consciousness (particularly to a Glasgow Coma Scale of <or= 11), brainstem signs (including pupillary changes, posturing, or irregular respirations), and a very recent seizure. The risk of not doing an LP when it is contraindicated because of concern of the risk of herniation is extremely small. In those considered high risk for herniation, interventions to control intracranial pressure, such as attention to airway, breathing, and circulation, with a mannitol infusion and antibiotics started, should be the priorities, followed by an urgent CT scan and not an LP.

Urgent surgical intervention in pediatric patients with Chiari malformation Type I

Patients with Chiari malformation Type I (CM-I) most commonly present with chronic symptoms. A search of the current medical literature revealed scant information regarding acute presentations of CM-I in either pediatric or adult patients. The authors report on two children who presented with rapidly worsening neurological symptoms attributable to a previously undiagnosed CM-I. One patient became profoundly hypopneic with dysphagia and right hemiparesis over a less than 48-hour period. The second patient presented with a few days of worsening right hemiparesis, gait disturbance, and anisocoria. In addition to a CM-I, magnetic resonance imaging in the second patient revealed a holocord syrinx. Following urgent posterior fossa decompression, both patients rapidly improved in the 24 hours immediately following surgery and continued to improve in the subsequent weeks. Few reports detail acute symptoms due to CM-I and those that do exist almost exclusively involve adult patients. Although seemingly rare, the clinician should consider CM-I in the differential diagnosis in pediatric patients presenting with acute brainstem or long tract signs.

Isolated limb sensory disturbance accompanied with sudden deafness from vertebral artery dissection: A case report

A 52-year-old male presented with right sudden deafness and left isolated limb sensory disturbance following posterior neck pain. An examination revealed a mild degree of right sensory neural hearing loss and decreased pain and thermal sensation in the left side below the L3 level. Brain MRI showed a small lesion in the midlateral portion of the right middle-rostral medulla. The combination of the image findings suggests a right lateral medullary infarction caused by a vertebral artery (VA) dissection. In our case, the fibers carrying the modalities of the lateral spinothalamic tract restricted to below the L3 level, sparing other sensations, suggest a selective involvement. This finding suggests that sensory dermatomal representation in the modality may be preserved at least up to the level of the medulla oblongata. Moreover, in our case, the small emboli originating from the dissected VA possibly caused a small selective cochlear infarction. This type of embolism may give rise to various combinations of symptoms depending on the branches involved. For patients with an acute onset of headache or neck pain who present with isolated limb sensory disturbance accompanied by sudden deafness, even without the classical brainstem signs, a VA dissection and medullary infarction should therefore be considered.

Cerebrotendinous xanthomatosis: case report with evidence of oxidative stress

Cerebrotendinous xanthomatosis is an autosomal recessive disorder of bile acid synthesis, characterized by mutation in the mitochondrial enzyme 27-hydroxylase that leads to an accumulation of cholestanol and cholesterol. Characterized clinically by premature bilateral cataracts, slowly progressive neurological deterioration with dementia, cerebellar and brainstem signs, peripheral neuropathy, and seizures, the disease presents pathologically with lipid granulomata with foamy histiocytes and cholesterol clefts. Replacement therapy with chenodeoxycholic acid slows progression of the disease but does not reverse neurological deficits. Here, we present the case of a 49-year-old woman diagnosed at autopsy with cerebrotendinous xanthomatosis, on the basis of bilateral Achilles tendon granulomas, and typical foamy histiocytic infiltration of the brain, most severe in the dentate nucleus, and a typical clinical presentation. To investigate the pathological manifestations of this disease further, we performed immunohistochemistry for Nϵ-(carboxymethyl)-lysine, an indicator of oxidative damage, and found strong labeling of cytoplasmic material within histiocytes. In summary, this case of undiagnosed cerebrotendinous xanthomatosis during life emphasizes the need for a greater awareness of the disease, and early diagnosis and treatment. Further, the involvement of oxidative stress in cerebrotendinous xanthomatosis indicates that combined therapy with chenodeoxycholic acid and antioxidants may improve clinical outcome.