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Cirugia Y Cirujanos 2020Wallenberg syndrome, or lateral medullar syndrome, is the clinical presentation of the infarct in the territory of posterior inferior cerebellar artery. Its signs and... (Review)
Review
Wallenberg syndrome, or lateral medullar syndrome, is the clinical presentation of the infarct in the territory of posterior inferior cerebellar artery. Its signs and symptoms include vertigo, nystagmus, diplopia, ipsilateral Horner syndrome, facial ruddiness and dry skin, dysphonia, dysphagia, dysarthria, ipsilateral loss of gag reflex, ipsilateral ataxia, ipsilateral impaired taste, ipsilateral facial pain and paresthesia, decreased ipsilateral blink reflex, contralateral hypoalgesia and thermoanaesthesia in the trunk and limbs; and ipsilateral facial hypoalgesia and thermoanaesthesia. Neuroanatomical knowledge is essential to its comprehension, study and diagnosis, because the classic neurological manifestations are easy to explain and understand if function and localization of affected anatomical structures are known as if the posterior cerebral circulation is.
Topics: Cerebellum; Cerebral Infarction; Deglutition Disorders; Dysphonia; Facial Pain; Horner Syndrome; Humans; Lateral Medullary Syndrome; Medulla Oblongata; Nystagmus, Pathologic; Reflex, Abnormal; Sensation Disorders; Vertigo
PubMed: 32539005
DOI: 10.24875/CIRU.19000801 -
The Canadian Veterinary Journal = La... Jan 2019Horner's syndrome arises from dysfunction of the oculosympathetic pathway and is characterized by miosis, enophthalmos, protrusion of the third eyelid, and ptosis. It... (Review)
Review
Horner's syndrome arises from dysfunction of the oculosympathetic pathway and is characterized by miosis, enophthalmos, protrusion of the third eyelid, and ptosis. It has been recognized in a wide variety of breeds and ages in small animal patients. The oculosympathetic pathway is a 3-neuron pathway. The central/first order neuron arises from the hypothalamus and extends down the spinal cord. The preganglionic/second order neuron arises from the first 3 thoracic spinal cord segments and travels through the thorax and cervical region until it synapses at the cranial cervical ganglion. The postganglionic/third order neuron travels from this ganglion to the orbit. Topical application of cocaine is the gold standard for differentiating Horner's syndrome from other causes of miosis. Topical 1% phenylephrine allows for identification of a post-ganglion Horner's syndrome. Numerous etiologies have been reported for Horner's syndrome, but idiopathic disease is most common. Ancillary diagnostics include otoscopic examination, thoracic radiographs, or advanced imaging. Treatment and prognosis are determined by the etiology.
Topics: Animals; Cat Diseases; Cats; Cocaine; Dog Diseases; Dogs; Horner Syndrome; Ophthalmic Solutions
PubMed: 30651655
DOI: No ID Found -
Brain : a Journal of Neurology Dec 2016Neuronal intranuclear inclusion disease (NIID) is a slowly progressive neurodegenerative disease characterized by eosinophilic hyaline intranuclear inclusions in the...
Neuronal intranuclear inclusion disease (NIID) is a slowly progressive neurodegenerative disease characterized by eosinophilic hyaline intranuclear inclusions in the central and peripheral nervous system, and also in the visceral organs. NIID has been considered to be a heterogeneous disease because of the highly variable clinical manifestations, and ante-mortem diagnosis has been difficult. However, since we reported the usefulness of skin biopsy for the diagnosis of NIID, the number of NIID diagnoses has increased, in particular adult-onset NIID. In this study, we studied 57 cases of adult-onset NIID and described their clinical and pathological features. We analysed both NIID cases diagnosed by post-mortem dissection and by ante-mortem skin biopsy based on the presence of characteristic eosinophilic, hyaline and ubiquitin-positive intanuclear inclusion: 38 sporadic cases and 19 familial cases, from six families. In the sporadic NIID cases with onset age from 51 to 76, dementia was the most prominent initial symptom (94.7%) as designated 'dementia dominant group', followed by miosis, ataxia and unconsciousness. Muscle weakness and sensory disturbance were also observed. It was observed that, in familial NIID cases with onset age less than 40 years, muscle weakness was seen most frequently (100%), as designated 'limb weakness group', followed by sensory disturbance, miosis, bladder dysfunction, and dementia. In familial cases with more than 40 years of onset age, dementia was most prominent (100%). Elevated cerebrospinal fluid protein and abnormal nerve conduction were frequently observed in both sporadic and familial NIID cases. Head magnetic resonance imaging showed high intensity signal in corticomedullary junction in diffusion-weighted image in both sporadic and familial NIID cases, a strong clue to the diagnosis. All of the dementia dominant cases presented with this type of leukoencephalopathy on head magnetic resonance imaging. Both sporadic and familial NIID cases presented with a decline in Mini-Mental State Examination and Frontal Assessment Battery scores. Based on these clinicopathological features, we proposed a diagnosis flow chart of adult-onset NIID. Our study suggested that the prevalence rate of adult-onset NIID may be higher than previously thought, and that NIID may be underdiagnosed. We should take NIID into account for differential diagnosis of leukoencephalopathy and neuropathy.
Topics: Adolescent; Adult; Age of Onset; Aged; Dementia; Female; Humans; Intranuclear Inclusion Bodies; Magnetic Resonance Imaging; Male; Middle Aged; Muscle Weakness; Neurodegenerative Diseases; Pedigree; Young Adult
PubMed: 27797808
DOI: 10.1093/brain/aww249 -
Brain Sciences Nov 2021Parinaud's syndrome involves dysfunction of the structures of the dorsal midbrain. We investigated the pathophysiology related to the signs and symptoms to better... (Review)
Review
Parinaud's syndrome involves dysfunction of the structures of the dorsal midbrain. We investigated the pathophysiology related to the signs and symptoms to better understand the symptoms of Parinaud's syndrome: diplopia, blurred vision, visual field defects, ptosis, squint, and ataxia, and Parinaud's main signs of upward gaze paralysis, upper eyelid retraction, convergence retraction nystagmus (CRN), and pseudo-Argyll Robertson pupils. In upward gaze palsy, three structures are disrupted: the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF), interstitial nucleus of Cajal (iNC), and the posterior commissure. In CRN, there is a continuous discharge of the medial rectus muscle because of the lack of inhibition of supranuclear fibers. In Collier's sign, the posterior commissure and the iNC are mainly involved. In the vicinity of the iNC, there are two essential groups of cells, the M-group cells and central caudal nuclear (CCN) group cells, which are important for vertical gaze, and eyelid control. Overstimulation of the M group of cells and increased firing rate of the CCN group causing eyelid retraction. External compression of the posterior commissure, and pretectal area causes pseudo-Argyll Robertson pupils. Pseudo-Argyll Robertson pupils constrict to accommodation and have a slight response to light (miosis) as opposed to Argyll Robertson pupils were there is no response to a light stimulus. In Parinaud's syndrome patients conserve a slight response to light because an additional pathway to a pupillary light response that involves attention to a conscious bright/dark stimulus. Diplopia is mainly due to involvement of the trochlear nerve (IVth cranial nerve. Blurry vision is related to accommodation problems, while the visual field defects are a consequence of chronic papilledema that causes optic neuropathy. Ptosis in Parinaud's syndrome is caused by damage to the oculomotor nerve, mainly the levator palpebrae portion. We did not find a reasonable explanation for squint. Finally, ataxia is caused by compression of the superior cerebellar peduncle.
PubMed: 34827468
DOI: 10.3390/brainsci11111469