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Brain Stem Disorder


Presentation

  • Eight of them (12%) presented with intractable nausea and vomiting as the sole initial symptom.[mdedge.com]
  • Abstract Three siblings presented with a progressive neurological disorder beginning in the third decade of life and characterised by palatal myoclonus, nystagmus, bulbar weakness and spastic tetraparesis.[jnnp.bmj.com]
  • Lateral tegmental hemorrhages present with 1 1/2 syndrome, small reactive pupils, limb ataxia of the cerebellar type, and contralateral hemisensory loss (Caplan and Goodwin, 1982). Diagnosis may be made via MRI or CT scan.[tchain.com]
  • This presents in childhood (average age at diagnosis of 10 years) and is preceded by jaundice and hepatosplenomegaly at birth in about half of patients; vertical gaze palsy is present in 80% of patients (32).[neuroophthalmology.ca]
  • In the patient whose images are shown above, there is a very rapid but small amplitude horizontal nystagmus, apparently pendular, not always present, superimposed on a constantly present nystagmus with fast phases going up and twisting to the right side[dizziness-and-balance.com]
Diplopia
  • A 51-year-old woman with MS developed acute vertigo, ataxia, diplopia, dysarthria, and bifacial weakness. She had been in clinical remission for 24 years without immunomodulatory therapy.[neurology.org]
  • There is a clinical continuum between BBE and Fisher's syndrome. [ 1 ] Bickerstaff reported eight patients who, in addition to acute ophthalmoplegia (diplopia) and ataxia, showed drowsiness, extensor plantar responses or hemisensory loss. [ 2 ] Epidemiology[patient.info]
  • Patients typically present subacutely with a wide variety of signs and symptoms dominated by 1 : cranial nerve dysfunction (e.g. dysarthria, altered facial sensation, diplopia) cerebellar signs (e.g. ataxia) long tract signs (e.g. spasticity, altered[radiopaedia.org]
  • Your doctor or therapist may also recommend corrective lenses or an eye patch to help manage diplopia. And hearing problems may necessitate the use of a hearing aid.[verywell.com]
  • Myasthenia gravis can also mimic a gaze palsy, though this is usually an asymmetric disease with complaints of diplopia (66). Variability and fatigability are key aspects that point to a neuromuscular junction problem.[neuroophthalmology.ca]
Unilateral Ptosis
  • Patients often have a Horner's syndrome (unilateral ptosis, miosis and facial anhidrosis). There also may be saccadic dysmetria (overshoot), saccadic pulsion (pulling of the eye during vertical saccades toward the side of lesion).[tchain.com]
  • However, a lateral fascicular III lesion affecting the superior recti, inferior oblique and levator may cause a monocular elevator palsy with unilateral ptosis (51).[neuroophthalmology.ca]
Strabismus
  • J Pediatr Ophthalmol Strabismus 1992;29:142-149. 50. Cadera W, Bloom JN, Karlik S, Viirre E. A magnetic resonance imaging study of double elevator palsy. Can J Ophthalmol 1997;32:250-253. 51. Hriso E, Masdeu JC, Miller A.[neuroophthalmology.ca]
Stereotyped Behavior
  • Evidence for a cerebellar role in reduced exploration and stereotyped behavior in autism. Biological Psychiatry, 49(8), 655–664. Scahill, L., Dimitropoulos, A., McDougle, C. J., Aman, M. G., Feurer, I. D., McCracken, J. T., et al. (2014).[frontiersin.org]
Compulsive Behavior
  • Keywords: Autism spectrum disorder (ASD), Obsessive-compulsive disorder (OCD), Transdiagnostic, Compulsive Behavior, Vertex analysis Conference: The Cerebellum inside out: cells, circuits and functions, ERICE (Trapani), Italy, 1 Dec - 5 Dec, 2016.[frontiersin.org]
Urinary Retention
  • Urinary retention, constipation, and weakness of the extremities accompanied by dysesthesias and ataxia soon ensued.[mdedge.com]
  • Signs of cholinergic autonomic hypofunction such as dilated unreactive pupils, urinary retention, and decreased bowel sounds are important clues (70).[neuroophthalmology.ca]
Vertigo
  • Vertigo is a common early symptom of brainstem strokes.[tchain.com]
  • However, because strokes are much less common than other sources of vertigo such as ear disorders, vertigo is only caused by central nervous system problems (including stroke) about 5% of the time. Migraine is a common cause of vascular vertigo.[dizziness-and-balance.com]
  • A 51-year-old woman with MS developed acute vertigo, ataxia, diplopia, dysarthria, and bifacial weakness. She had been in clinical remission for 24 years without immunomodulatory therapy.[neurology.org]
  • Peripheral vertigo; Central vertigo; Dizziness; Benign positional vertigo; Benign paroxysmal positional vertigo Bhattacharyya N, Gubbels SP, Schwartz SR, et al. Clinical practice guideline: benign paroxysmal positional vertigo (update).[medlineplus.gov]
  • The vertigo arising from central lesions tends to be more chronic and debilitating than that resulting from peripheral vestibulopathy. Vertigo of central origin often becomes unremitting and disabling. There may be nausea and vomiting.[healthcentral.com]
Dizziness
  • A person may have vertigo, dizziness and severe imbalance without the hallmark of most strokes — weakness on one side of the body. The symptoms of vertigo dizziness or imbalance usually occur together; dizziness alone is not a sign of stroke.[strokeassociation.org]
  • Vertigo is a sensation of motion or spinning that is often described as dizziness. Vertigo is not the same as being lightheaded. People with vertigo feel as though they are actually spinning or moving, or that the world is spinning around them.[medlineplus.gov]
  • Definition Deficits in vestibular function typically cause dizziness, loss of balance, and diminished functional independence and reflect a disturbance of the central vestibular pathways in the brain.[healthcentral.com]
  • Most commonly, people have neck pain and headache, but if the spinal cord or lowest part of the brain (brain stem) is affected, people may have difficulty sensing vibration, pain, and temperature and may have weak muscles, dizziness, and impaired vision[merckmanuals.com]
  • Dizziness and problems with balance and coordination Swallowing difficulties Sleep apnea Most children born with type II Chiari malformation have hydrocephalus.[webmd.com]
Nystagmus
  • In the author's experience, these patients often exhibit rebound nystagmus, which is a variant of gaze-evoked nystagmus. Some of these patients have upbeating nystagmus supine, often confused with BPPV.[dizziness-and-balance.com]
  • In the author's experience, these patients often exhibit rebound nystagmus, which is a variant of gaze-evoked nystagmus .[tchain.com]
  • Abstract Three siblings presented with a progressive neurological disorder beginning in the third decade of life and characterised by palatal myoclonus, nystagmus, bulbar weakness and spastic tetraparesis.[jnnp.bmj.com]
  • A classic sign is convergence-retraction nystagmus. (see case 51 ) Fragments of the pretectal syndrome are common.[neuroophthalmology.ca]
  • Medial inferior pontine syndrome (occlusion of paramedian branch of basilar artery) On side of lesion • Paralysis of conjugate gaze to side of lesion (preservation of convergence): Center for conjugate lateral gaze(PPRF) • Nystagmus: Vestibular nucleus[slideshare.net]
Dysarthria
  • A 51-year-old woman with MS developed acute vertigo, ataxia, diplopia, dysarthria, and bifacial weakness. She had been in clinical remission for 24 years without immunomodulatory therapy.[neurology.org]
  • It is typified by vertigo, ipsilateral hemiataxia, dysarthria, ptosis and miosis. Most patients with this stroke recover very well and often resume their previous activities (Nelles et al, 1998).[tchain.com]
  • Patients typically present subacutely with a wide variety of signs and symptoms dominated by 1 : cranial nerve dysfunction (e.g. dysarthria, altered facial sensation, diplopia) cerebellar signs (e.g. ataxia) long tract signs (e.g. spasticity, altered[radiopaedia.org]
  • Another rare autosomal recessive condition consists of cerebellar ataxia and dysarthria, myoclonus and seizures, sensory neuropathy and upgaze palsy (34).[neuroophthalmology.ca]
  • In addition, people with migraine with brainstem aura get brainstem aura symptoms such as: Dysarthria (slurred speech) Vertigo (feeling of movement/spinning of self or environment) Tinnitus (ringing in ears) Hypacusis (impaired hearing) Diplopia (double[americanmigrainefoundation.org]
Upbeat Nystagmus
  • Some of these patients have upbeating nystagmus supine, often confused with BPPV. Individuals with midline pontine infarcts usually have normal ABR testing (Faught and Oh, 1985).[dizziness-and-balance.com]
  • Upbeat nystagmus may occur. Small vessel disease (diabetes, hypertension, hypercholesterolemia) is the usual cause. Pontine hyperintense lesions. It is fairly common to encounter areas of increased signal on T2 MRI in the pons.[tchain.com]
  • Some have other signs such as impaired convergence (47), pupillary light-near dissociation, upbeat nystagmus in upgaze (48), and, rarely, contralateral ptosis.[neuroophthalmology.ca]

Workup

Candida
  • Candida Fink, MD (New Rochelle, New York) is a Board Certified Adult, Child, and Adolescent Psychiatrist in private practice in the New York area. Dr.[books.google.com]

Treatment

  • There is no treatment.[msdvetmanual.com]
  • Fink grew up with mood disorders in her family, so she has seen them from every angle and knows the importance of mobilizing the family as a part of any successful treatment.[books.google.com]
  • In addition, the center continues to expand and pioneer treatments directed at other abnormalities in and around the brainstem.[neurosurgery.pitt.edu]
  • Treatment with plasmapheresis and methylprednisolone generally relieved gait and sensory complaints and improved bladder and bowel function as well as the nausea and vomiting.[mdedge.com]

Prognosis

  • Prognosis is generally quite good with full or near full recovery expected at 6 months. Diagnosis is generally via MRI.[tchain.com]
  • Prognosis of the pretectal syndrome varies with cause. Some resolution can be expected over several weeks as thalamic hemorrhages resolve. Shunting in hydrocephalus has variable effect (36). 5.[neuroophthalmology.ca]
  • . • MRI may provide further detail and aid prognosis. • Surgery is reserved for select cases in which specific expertise is available.[medlink.com]
  • Treatment and prognosis Patients typically respond rapidly to the administration of glucocorticosteroids but have a tendency to relapse if immunosuppression is ceased. As such long-term immunosuppression is recommended 1.[radiopaedia.org]
  • Did you know posterior fossa lesions in MS portend a poor prognosis? #MSBlog #MSResearch #ClinicSpeak "The brainstem is the part of the central nervous system (CNS) between the spinal cord and so called cerebral hemispheres.[multiple-sclerosis-research.blogspot.com]

Etiology

  • Furthermore, the concept of secondary mania casts doubt on any unitary or single-agent hypothesis of the etiology of mania and supports the notion of a continuum of psychopathologic syndromes.[jamanetwork.com]
  • MRI is frequently needed to make a specific diagnosis and to separate vascular etiologies from tumor and other structural injuries. The pattern of sensory disturbance may be helpful.[tchain.com]
  • Etiology These syndromes are classically caused by ischemic strokes that occur secondary to occlusion of small perforating arteries of the posterior circulation, however many have also been described secondary to hemorrhage, neoplasm, and demyelination[radiopaedia.org]
  • However, the condition is still poorly understood and, based on its natural history, an (auto)immune-mediated or other inflammatory pathogenesis of unknown etiology is proposed 1,4.[radiopaedia.org]
  • Etiology Several different vascular territories exist within the thalamus, giving rise to distinct families of thalamic stroke presentations (7, 8).[neuroophthalmology.ca]

Epidemiology

  • Basilar-type migraine: clinical, epidemiologic, and genetic features. Neurology. 2006;66(6):880-886. Ying G, Fan W, Li N, et al. Clinical characteristics of basilar-type migraine in the neurological clinic of a university hospital. Pain Medicine.[migraine.com]
  • Epidemiology and Genetics of Parkinson's Disease Walter A.[mayo.edu]
  • There is a clinical continuum between BBE and Fisher's syndrome. [ 1 ] Bickerstaff reported eight patients who, in addition to acute ophthalmoplegia (diplopia) and ataxia, showed drowsiness, extensor plantar responses or hemisensory loss. [ 2 ] Epidemiology[patient.info]
  • Basilar-type migraine: Clinical, epidemiologic, and genetic features. Neurology 2006;66: 880-886. Klapper J, Mathew N, Nett R. Triptans in the treatment of basilar migraine and migraine with prolonged aura. Headache. 2001;41:981-984.[americanmigrainefoundation.org]
  • […] allow trainees to eventually return to their country of origin and to facilitate translation of these rapidly emerging experimental technologies into novel therapeutic applications and into creative collaborative partnerships in the areas of genetic epidemiology[einstein.yu.edu]
Sex distribution
Age distribution

Pathophysiology

  • This finding has implications for pathophysiologic models of panic disorder, and provides structural evidence for the role of the brainstem in neurocircuitry models of panic disorder.[nyuscholars.nyu.edu]
  • This finding has implications for pathophysiologic models of panic disorder, and provides structural evidence for the role of the brainstem in neurocircuitry models of panic disorder. a Functional Neuroimaging Laboratory, Department of Psychiatry, Weill[journals.lww.com]
  • In Barnet HJM (and others, Eds), Stroke: Pathophysiology, Diagnosis and Management. New York: Chrchill-Livingstone, pp 549-619, 1986 Dai, A. I. and M. Wasay (2006). "Wernekink comissure syndrome: a rare midbrain syndrome secondary to stroke."[dizziness-and-balance.com]

Prevention

  • Prevention of MS brainstem problems relies on the same disease-modifying treatments (DMTs) used to prevent MS progression.[verywell.com]
  • Of the preventive medications, topiramate, verapamil and lamotrigine are most commonly used.[americanmigrainefoundation.org]
  • Peever hopes that his research will pave the way for neuroprotective therapies that would prevent against the development of such neurodegenerative disorders.[medicalnewstoday.com]
  • These studies will contribute greatly to understanding the causes and possible prevention of PD by exploring novel hypotheses and by using innovative methods.[mayo.edu]
  • Exercises can also strengthen your muscles to help prevent falls. To prevent worsening of symptoms during an episode of vertigo, try the following: Keep still. Sit or lie down when symptoms occur. Gradually resume activity.[medlineplus.gov]

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