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Spinal Cord Disease
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
LMN (nuclear or infranuclear) signs: Muscle wasting.Fasciculations.Muscle weakness.Areflexia.
Unexplained Fever In Neurological Disorders
Published in Benedict Isaac, Serge Kernbaum, Michael Burke, Unexplained Fever, 2019
Although it is not difficult to diagnose rabies when the classical clinical signs described by Trousseau are present in an individual bitten by a rabid animal, this diagnosis is sometimes tricky and may be overlooked for several reasons: (a) incubation period may last from 15 days to 1 year; (b) during the last several years only 50% of patients in the U. S. with rabies have had a history of a bite; (c) following a prodrome of fever, headache, malaise, and anorexia, in half of the patients a nonspecific clinical picture of encephalitis might be present; (d) in 5 to 20% of cases ascending paralysis with areflexia similar to the Guillain-Barre syndrome (GBS) may be present.37 This clinical presentation known also as “dumb-rabies” is seen mainly when transmitted by infected bats. The spinal fluid shows pleocytosis only in 25% of patients but protein content is usually elevated, consistent with the classical albuminocytological dissociation seen in GBS, thus increasing the chances of completely overlooking the possibility of rabies.
Shy-Drager Syndrome and Multiple System Atrophy
Published in David Robertson, Italo Biaggioni, Disorders of the Autonomic Nervous System, 2019
Application of urodynamic and electromyographic strategies for investigating bladder function in patients with MSA has yielded several important observations. Voluntary and involuntary aspects of control appear to be affected (Kirby et al., 1986). Patients are unable to initiate a micturition reflex. Bladder filling causes involuntary detrusor contractions. In addition, MSA patients also develop severe urethral dysfunction, including a loss of urethral tone. Abnormal motor units recorded from the striated part of the urethral sphincter suggest reinnervation. The pattern of involvement may vary, e.g. some patients have detrusor areflexia while others are hyperreflexic (Salinas et al., 1986). These abnormalities reflect the pandysautonomia in MSA which in this case results primarily from selective involvement of sacral anterior horn cells (Onuf’s nucleus).
Decision-making and challenges within the evolving treatment algorithm in spinal muscular atrophy: a clinical perspective
Published in Expert Review of Neurotherapeutics, 2023
Lakshmi Balaji, Michelle A Farrar, Arlene M D’Silva, Didu S Kariyawasam
Ambiguous or subtle clinical findings may sometimes be apparent, denoting a prodromal period, or phenotransition from pre-symptomatic to clinically manifest, for affected individuals diagnosed through NBS [65]. For example, vascular dysautonomia, a rare finding that may precede weakness or areflexia, may be a clinical manifestation of SMA in neonates [70]. Hyper-reflexia may precede areflexia. Phenoconversion may be rapid for neonates with 2SMN2 copies, with a normal compound muscle action potential (CMAP) early on that precipitously drops and reaches a nadir within a few weeks [71]. Significantly, approximately 40% of those with 2SMN2 copies manifest symptoms of SMA within 6 postnatal weeks [67]. While this population has not been well studied within clinical trials, recent data has shown individuals with clinically manifest SMA diagnosed following NBS may achieve assisted or independent ambulation [67]. Compared to individuals with clinically based diagnoses and a longer latency to initiation of disease-modifying therapies, improved outcomes have been observed, suggesting a neuromuscular penumbra amenable to urgent therapy [67,72,73].
Ataxia and ophthalmoplegia: an atypical case of Miller Fisher syndrome (MFS) with anti-GAD antibody
Published in International Journal of Neuroscience, 2022
Ali R. Shoraka, Xiang Fang, Diaa Hamouda, Bhanu Gogia, Xiangping Li
Miller Fisher syndrome (MFS) is a rare variant of Guillain-Barre syndrome (GBS) which was first described by James Collier in 1932 as a triad of symptoms including ophthalmoplegia, ataxia, and areflexia. Later in 1956, Miller Fisher characterized and classified it as a unique entity within GBS spectrum [1]. There are incomplete or atypical forms of MFS as well which include acute ataxic neuropathy in the absence of ophthalmoplegia and acute ophthalmoparesis in the absence of ataxia [2,3]. An areflexia is present in about 80% of cases [4]. MFS is an immune-mediated disease and specific anti-ganglioside antibodies, specifically anti-GQ1b antibodies, are detected between 85% and 90% of the patients [5]. This association was first described in 1992 by Chiba et al. [3]. However, a minority of patients (10%−15%) are GQ1b-seronagative [6]. Among MFS patients with negative GQ1b antibody, there are two reported cases of high titer anti-glutamic acid decarboxylase antibody (GAD-Ab) [5,7]. In this article, we describe an atypical GQ1b seronegative Miller Fisher syndrome (MFS) case with elevated GAD-Ab, Voltage-Gated Calcium Channel antibody (VGCC-Ab), and antibodies to thyroid peroxidase (TPO-Ab) and thyroglobulin (Tg-Ab).
Acute motor-sensory axonal neuropathy associated with systemic lupus erythematosus
Published in Baylor University Medical Center Proceedings, 2019
Smathorn Thakolwiboon, Amputch Karukote, Gyeongmo Sohn
A previously healthy 72-year-old white man presented with rapidly progressive bilateral ascending flaccid weakness and tingling sensation in all four extremities. He became quadriplegic within 3 days. Additionally, joint swelling, pain, tenderness, and morning stiffness were reported in both knees and finger joints. He denied dyspnea, voice change, and swallowing difficulty. He had no history of recent fever, diarrhea, or vaccination. At presentation, he was afebrile (98.8°F) and normotensive (126/65 mm Hg). He had no rash, hair loss, or oral ulcer. There was no superficial lymphadenopathy. Pain with passive movement and tenderness were noted in both knees and joints of the fingers. Neurological examination showed complete quadriplegia, hypotonia, and areflexia. Hyperesthesia was noted in all extremities. Cranial nerves were intact.