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Neurology India 2022"Thirteen-and-a-half" is a newly described clinical syndrome characterized by the combination of the one-and-a-half syndrome with fifth and seventh cranial nerve nuclei...
BACKGROUND
"Thirteen-and-a-half" is a newly described clinical syndrome characterized by the combination of the one-and-a-half syndrome with fifth and seventh cranial nerve nuclei involvement (1 + 5 + 7 = 13). To the authors' knowledge, this is the first report of the thirteen-and-a-half syndrome secondary to pontine cavernoma and, overall, only the second reported case of this syndrome in the literature till date.
CASE REPORT
A 20-year-old man presented with the clinical features suggestive of the thirteen-and-a-half syndrome, explained radiologically by pontine cavernoma. We operated him using a suboccipital transvermian approach and he is doing well at 2.5 years follow-up. Interestingly, his one-and-a-half syndrome has partially improved to left horizontal gaze palsy.
CONCLUSION
The clinical appreciation of the thirteen-and-a-half syndrome precisely localizes the lesion to ipsilateral dorsal pontine tegmentum. Neurosurgeons must be aware of the newly described "one-and-a-half- plus" syndromes as they help in a better understanding of pathoanatomy caused by different disease processes in the brainstem.
Topics: Adult; Brain Stem; Hemangioma, Cavernous; Humans; Male; Pons; Strabismus; Syndrome; Young Adult
PubMed: 35263913
DOI: 10.4103/0028-3886.338728 -
Journal of Neurology Dec 2022Vertical pendular nystagmus (PN) rarely occurs with acute pontine lesions. To hypothesize a pathophysiology for acute vertical PN, we analyzed the clinical... (Review)
Review
Vertical pendular nystagmus (PN) rarely occurs with acute pontine lesions. To hypothesize a pathophysiology for acute vertical PN, we analyzed the clinical characteristics and quantitative eye-movement recordings of one new case with acute vertical PN and an additional 11 patients from the literature. Most patients had extensive pontine lesions causing either the locked-in syndrome or unresponsiveness, but two conscious patients had focal lesions restricted to the paramedian caudal pontine tegmentum. All patients presented a complete or partial horizontal gaze palsy, and about half showed ocular bobbing before or during the appearance of vertical PN. The vertical oscillations were conjugate at a frequency of 1-5 Hz, and the amplitudes were variable, ranging from 0.2° to 40°. The peak velocities were asymmetric in some patients, faster with downward movements. About half of the patients developed palatal tremor several weeks or months after presenting with acute vertical PN. Based on the location of the lesions and results of eye-movement recordings, we suggest two possible mechanisms for acute vertical PN; oscillations originating in the inferior olives due to disruption of the central tegmental tract or low-velocity saccadic oscillations caused by omnipause neuron damage.
Topics: Humans; Nystagmus, Pathologic; Eye Movements; Pons; Ocular Motility Disorders; Movement
PubMed: 35904591
DOI: 10.1007/s00415-022-11314-5 -
AJNR. American Journal of Neuroradiology May 2021Studies associate repeat gadolinium-based contrast agent administration with T1 shortening in the dentate nucleus and globus pallidus, indicating CNS gadolinium...
BACKGROUND AND PURPOSE
Studies associate repeat gadolinium-based contrast agent administration with T1 shortening in the dentate nucleus and globus pallidus, indicating CNS gadolinium deposition, most strongly with linear agents but also reportedly with macrocyclics. Renal impairment effects on long-term CNS gadolinium deposition remain underexplored. We investigated the relationship between signal intensity changes and renal function in patients who received ≥10 administrations of the macrocyclic agent gadobutrol.
MATERIALS AND METHODS
Patients who underwent ≥10 brain MR imaging examinations with administration of intravenous gadobutrol between February 1, 2014, and January 1, 2018, were included in this retrospective study. Dentate nucleus-to-pons and globus pallidus-to-thalamus signal intensity ratios were calculated, and correlations were calculated between the estimated glomerular filtration rate (minimum and mean) and the percentage change in signal intensity ratios from the first to last scan. Partial correlations were calculated to control for potential confounders.
RESULTS
One hundred thirty-one patients (73 women; mean age at last scan, 55.9 years) showed a mean percentage change of the dentate nucleus-to-pons of 0.31%, a mean percentage change of the globus pallidus-to-thalamus of 0.15%, a mean minimum estimated glomerular filtration rate of 69.65 (range, 10.16-132.26), and a mean average estimated glomerular filtration rate at 89.48 (range, 38.24-145.93). No significant association was found between the estimated glomerular filtration rate and percentage change of the dentate nucleus-to-pons (minimum estimated glomerular filtration rate, = -0.09, = .28; average estimated glomerular filtration rate, = -0.09, = .30,) or percentage change of the globus pallidus-to-thalamus ( = 0.07, = .43; = 0.07, = .40). When we controlled for age, sex, number of scans, and total dose, there were no significant associations between the estimated glomerular filtration rate and the percentage change of the dentate nucleus-to-pons ( = 0.16, = .07; = 0.15, = .08) or percentage change of the globus pallidus-to-thalamus ( = -0.14, = .12; = -0.15, = .09).
CONCLUSIONS
In patients receiving an average of 12 intravenous gadobutrol administrations, no correlation was found between renal function and signal intensity ratio changes, even in those with mild or moderate renal impairment.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Central Nervous System; Cerebellar Nuclei; Child; Child, Preschool; Contrast Media; Female; Gadolinium; Glomerular Filtration Rate; Humans; Kidney; Kidney Diseases; Kidney Function Tests; Magnetic Resonance Imaging; Male; Middle Aged; Organometallic Compounds; Pons; Retrospective Studies; Young Adult
PubMed: 33632738
DOI: 10.3174/ajnr.A7031 -
Journal of Neurophysiology Jul 2022Deiters' neurons, located exclusively in the lateral vestibular nucleus (LVN), are involved in vestibulospinal reflexes, innervate extensor motoneurons that drive...
Deiters' neurons, located exclusively in the lateral vestibular nucleus (LVN), are involved in vestibulospinal reflexes, innervate extensor motoneurons that drive antigravity muscles, and receive inhibitory inputs from the cerebellum. We investigated intrinsic membrane properties, short-term plasticity, and inhibitory synaptic inputs of mouse Deiters' and non-Deiters' neurons within the LVN. Deiters' neurons are distinguished from non-Deiters' neurons by their very low input resistance (105.8 vs. 521.8 MΩ, respectively), long axons that project as far as the ipsilateral lumbar spinal cord, and expression of the cytostructural protein nonphosphorylated neurofilament protein (NPNFP). Whole cell patch-clamp recordings in brain stem slices show that most Deiters' and non-Deiters' neurons were tonically active (>92%). Short-term plasticity was studied by examining discharge rate modulation following release from hyperpolarization [postinhibitory rebound firing (PRF)] and depolarization [firing rate adaptation (FRA)]. PRF and FRA gain were similar in Deiters' and non-Deiters' neurons (PRF 24.9 vs. 20.2 Hz and FRA gain 231.5 vs. 287.8 spikes/s/nA, respectively). Inhibitory synaptic input to both populations showed that GABAergic rather than glycinergic inhibition dominated. However, GABA miniature inhibitory postsynaptic current (mIPSC) frequency was much higher in Deiters' neurons compared with non-Deiters' neurons (∼15.9 vs. 1.4 Hz, respectively). Our data suggest that Deiters' neurons can be reliably identified by their intrinsic membrane and synaptic properties. They are tonically active and glutamatergic, have low sensitivity or "gain," exhibit little adaptation, and receive strong GABAergic input. Deiters' neurons also have minimal short-term plasticity, and together these features suggest they are well suited to a role in encoding tonic signals for the vestibulospinal reflex. Deiters' neurons within the lateral vestibular nucleus project the length of the spinal cord and activate antigravity extensor muscles. Deiters' neurons were characterized anatomically and physiologically in mice. Deiters' neurons are tonically active, have homogeneous intrinsic membrane properties, including low input resistance, and receive significant GABAergic synaptic inputs. Deiters' neurons show little modulation in response to current injection. These features are consistent with Deiters' neurons responding to perturbations to maintain posture and balance.
Topics: Animals; Mice; Neurons; Spinal Cord; Synaptic Transmission; Vestibular Nuclei; Vestibular Nucleus, Lateral; gamma-Aminobutyric Acid
PubMed: 35730750
DOI: 10.1152/jn.00016.2022 -
Internal and Emergency Medicine Oct 2023
Topics: Humans; Myelinolysis, Central Pontine; Magnetic Resonance Imaging; Pons
PubMed: 37277602
DOI: 10.1007/s11739-023-03332-6 -
Molecular Brain May 2020The linear nucleus (Li) was identified in 1978 from its projections to the cerebellum. However, there is no systematic study of its connections with other areas of the...
The linear nucleus (Li) was identified in 1978 from its projections to the cerebellum. However, there is no systematic study of its connections with other areas of the central nervous system possibly due to the challenge of injecting retrograde tracers into this nucleus. The present study examines its afferents from some nuclei involved in motor and cardiovascular control with anterograde tracer injections. BDA injections into the central amygdaloid nucleus result in labeled fibers to the ipsilateral Li. Bilateral projections with an ipsilateral dominance were observed after injections in a) jointly the paralemniscal nucleus, the noradrenergic group 7/ Köllike -Fuse nucleus/subcoeruleus nucleus, b) the gigantocellular reticular nucleus, c) and the solitary nucleus/the parvicellular/intermediate reticular nucleus. Retrogradely labeled neurons were observed in Li after BDA injections into all these nuclei except the central amygdaloid and the paralemniscal nuclei. Our results suggest that Li is involved in a variety of physiological functions apart from motor and balance control it may exert via its cerebellar projections.
Topics: Afferent Pathways; Amygdala; Animals; Biotin; Cerebellum; Dextrans; Dorsal Raphe Nucleus; Medulla Oblongata; Mice; Mice, Inbred C57BL; Neural Pathways; Neurons; Pontine Tegmentum; Trigeminal Nuclei; Vestibular Nuclei
PubMed: 32370769
DOI: 10.1186/s13041-020-00602-8 -
Multiple Sclerosis and Related Disorders Jul 2020CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) is a chronic central nervous system (CNS) inflammatory disorder....
BACKGROUND
CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) is a chronic central nervous system (CNS) inflammatory disorder. It may be associated with lymphoma and macrophage activation, while the related report of histiocytes (macrophage) activation involved in pathogenesis of CLIPPERS is rare. We present the first "probable CLIPPERS" case associated with histiocytic sarcoma (HS) progressed to hemophagocytic syndrome (HPS) in a 38-year-old man patient.
CASE PRESENTATION
The 38-year-old man presented with facial numbness, diplopia, gait ataxia and glossolalia for 29 months. Brain MRI showed gadolinium enhancement peppering the pons and extending into the midbrain, medulla, brachium pontis, cerebellum and thalamus. The patient's CNS symptoms were improved significantly and accompanied by marked radiological improvement after glucocorticoids therapy, while the disease courses presented relapsing-remitting and glucocorticoids-dependent. Multiple nodules in the abdomen were accidentally discovered by the abdominal Computed tomography (CT) during the remission period. HS was diagnosed by histological examination of the abdominal node biopsy accompanied by CLIPPERS relapse, and eventually progressed to HPS.
CONCLUSIONS
CLIPPERS could be a syndrome of lymphohistiocytic disorders.
Topics: Adult; Brain Diseases; Histiocytic Sarcoma; Humans; Inflammation; Lymphohistiocytosis, Hemophagocytic; Male; Pons; Syndrome
PubMed: 32234602
DOI: 10.1016/j.msard.2020.102063 -
Multiple Sclerosis and Related Disorders Jan 2020Diagnosis of central or peripheral facial palsy (FP) is traditionally based on clinical evaluation. This study aims at investigating the relationship between clinical... (Observational Study)
Observational Study
BACKGROUND
Diagnosis of central or peripheral facial palsy (FP) is traditionally based on clinical evaluation. This study aims at investigating the relationship between clinical evaluation of FP and lesion location as visible on Magnetic Resonance Imaging (MRI) in patients with Multiple Sclerosis (MS) for the purpose of adding supporting evidence to the diagnosis of central or peripheral FP in these patients.
METHODS
A retrospective study was conducted on data from patients who underwent MS treatment between January 2016 and January 2019 at the MS Center of Wayne State University, MI, USA, and presented with at least one episode of FP during the observational period. The following data was collected from each patient: demographics, time from MS onset, side of FP, FP type (central or peripheral, as clinically evaluated), FP onset, FP treatment, amount of recovery of normal facial movements, time elapsed from beginning of FP treatment, number of FP recurrences, lesions presence/absence and location as visible on MRI. Correlation analysis was performed to assess to which extent clinical evaluation of FP correlated with presence of MRI lesions in different locations.
RESULTS
Eighteen patients were included in this study. In thirteen patients (72.2%) FP was classified as peripheral. Among them only five (38.4%) displayed one or more lesions in the pons. Correlation between presence of lesions in the pons and presence of peripheral FP was statistically significant (p = 0.02). Correlation between presence of lesions in the cortex (observed in 61.5% of patients with FP clinically evaluated as peripheral) and presence of FP clinically evaluated as peripheral was also statistically significant (p = 0.01). Finally, presence of lesions in the cortex was significantly correlated with presence of FP clinically evaluated as central (p = 0.02).
CONCLUSIONS
FP clinically classified as peripheral may be caused by central lesions in the cortex or pons and not only by peripheral facial nerve damage. In MS patients, FP may appear at the onset of the disease and be misdiagnosed as Bell 's palsy. Clinicians should carefully approach FP diagnosis and perform a brain as well as temporal bone MRI before pursuing pharmacological treatment.
Topics: Adult; Cerebral Cortex; Facial Nerve; Facial Paralysis; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Pons; Retrospective Studies; Young Adult
PubMed: 31670209
DOI: 10.1016/j.msard.2019.101456 -
Journal of Neurology Sep 2019Over the last few decades, several studies have been conducted to identify the mechanisms involved in spontaneous functional recovery following peripheral vestibular... (Comparative Study)
Comparative Study Review
Over the last few decades, several studies have been conducted to identify the mechanisms involved in spontaneous functional recovery following peripheral vestibular damage. Different reactive processes occur at both the central and peripheral levels over the first few hours after the loss of the peripheral vestibular input. The restoration of the electrophysiological homeostasis between opposite vestibular nuclei is one of the key mechanisms of central compensation. This is achieved through a mosaic of biochemical events within the vestibular nuclei that each occur with their own kinetics. At the same time, under specific conditions, strong synaptic plasticity may take place within the vestibular sensory organs. It is thought that this reactive plasticity can contribute to the repair of damaged contacts between hair cells and fibres of the vestibular nerve, thus gradually restoring peripheral sensory input. These different plastic phenomena seem to reproduce those observed during development. Research is now needed to identify the cellular and molecular mechanisms that support this spontaneous peripheral repair process, with the ambition 1 day to be able to control it and stimulate the restoration of gait and balance.
Topics: Adaptation, Physiological; Animals; Humans; Neuronal Plasticity; Recovery of Function; Synapses; Vestibular Nuclei; Vestibule, Labyrinth
PubMed: 31134376
DOI: 10.1007/s00415-019-09388-9 -
BMC Neurology Aug 2019The topographic location of acute pontine infarction is associated with clinical syndromes and prognosis. Previous studies focused on isolated pontine infarction, but...
BACKGROUND
The topographic location of acute pontine infarction is associated with clinical syndromes and prognosis. Previous studies focused on isolated pontine infarction, but the topographic location of unisolated pontine infarction has remained unclear.
METHODS
This was a prospective, multicenter, longitudinal registry study. Patients with acute pontine infarction confirmed by magnetic resonance imaging (MRI) were enrolled. Based on the territory of the pontine artery, the topographic location was divided into anteromedial, anterolateral, tegmental, bilateral and unilateral multiple infarctions.
RESULTS
From May 1, 2003, to Oct 31, 2017, 1003 patients were enrolled, and 330 had unisolated pontine infarction. For isolated pontine infarction, 44.9, 19.8, 16.0, 13.1 and 6.2% of patients had anteromedial, anterolateral, tegmental, bilateral and unilateral multiple pontine infarctions, respectively. For unisolated pontine infarction, 30.3, 19.7, 24.5, 15.2 and 10.3% of patients had anteromedial, anterolateral, tegmental, bilateral and unilateral multiple pontine infarctions, respectively.
CONCLUSION
In this large series study, our data revealed fewer anteromedial infarctions and more tegmental and unilateral multiple infarctions in patients with unisolated pontine infarction than in patients with isolated pontine infarction.
Topics: Adult; Aged; Brain Stem Infarctions; Female; Humans; Infarction; Male; Middle Aged; Pons; Prospective Studies
PubMed: 31382915
DOI: 10.1186/s12883-019-1411-6