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Neurology International is published by MDPI from Volume 12 Issue 3 (2020). Previous articles were published by another publisher in Open Access under a CC-BY licence, and they are hosted by MDPI on mdpi.com as a courtesy and upon agreement with PAGEPress.

Neurol. Int., Volume 5, Issue 2 (June 2013) – 6 articles

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631 KiB  
Case Report
Adult Mitochondrial DNA Depletion Syndrome with Mild Manifestations
by Josef Finsterer, Gabor G. Kovacs and Uwe Ahting
Neurol. Int. 2013, 5(2), e9; https://doi.org/10.4081/ni.2013.e9 - 25 Jun 2013
Cited by 6 | Viewed by 1
Abstract
Mitochondrial DNA depletion syndrome (MDS) is usually a severe disorder of infancy or childhood, due to a reduced copy number of mtDNA molecules. MDS with only mild, non-specific clinical manifestations and onset in adulthood has not been reported. A 47-year-old Caucasian female with [...] Read more.
Mitochondrial DNA depletion syndrome (MDS) is usually a severe disorder of infancy or childhood, due to a reduced copy number of mtDNA molecules. MDS with only mild, non-specific clinical manifestations and onset in adulthood has not been reported. A 47-year-old Caucasian female with short stature and a history of migraine, endometriosis, Crohn’s disease, C-cell carcinoma of the thyroid gland, and a family history positive for mitochondrial disorder (2 sisters, aunt, niece), developed day-time sleepiness, exercise intolerance, and myalgias in the lower-limb muscles since age 46y. She slept 9-10 hours during the night and 2 hours after lunch daily. Clinical exam revealed sore neck muscles, bilateral ptosis, and reduced Achilles tendon reflexes exclusively. Blood tests revealed hyperlipidemia exclusively. Nerve conduction studies, needle electromyography, and cerebral and spinal magnetic resonance imaging were non-informative. Muscle biopsy revealed detached lobulated fibers with subsarcolemmal accentuation of the NADH and SDH staining. Real-time polymerase chain reaction revealed depletion of the mtDNA down to 9% of normal. MDS may be associated with a mild phenotype in adults and may not significantly progress during the first year after onset. In an adult with hypersomnia, severe tiredness, exercise intolerance, and a family history positive for mitochondrial disorder, a MDS should be considered. Full article
806 KiB  
Case Report
A Discrepancy between Clinical Course and Magnetic Resonance Imaging in a Case of Non-Herpetic Acute Limbic Encephalitis
by Naoto Kohno, Yuko Kawakami, Chizuko Hamada, Genya Toyoda, Hirokazu Bokura, Shuhei Yamaguchi and Yukitoshi Takahashi
Neurol. Int. 2013, 5(2), e8; https://doi.org/10.4081/ni.2013.e8 - 25 Jun 2013
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Abstract
We report the case of a 64-year old man who presented memory disturbance, low-grade fever, weight loss, and bilateral hand tremors for three months. He was diagnosed with non-herpetic acute limbic encephalitis (NHALE). Follow-up magnetic resonance imaging (MRI) revealed new lesions after symptomatic [...] Read more.
We report the case of a 64-year old man who presented memory disturbance, low-grade fever, weight loss, and bilateral hand tremors for three months. He was diagnosed with non-herpetic acute limbic encephalitis (NHALE). Follow-up magnetic resonance imaging (MRI) revealed new lesions after symptomatic improvement following steroid pulse therapy. This may indicate that there is a time lag between the disturbance or recovery of neurons and astrocytes. Thus, other lesions might occasionally appear during convalescence in patients with NHALE, even if only minimal lesions were found on the initial MRI. Full article
541 KiB  
Case Report
A Rare Case of Infectious Mononucleosis Complicated by Guillain-Barre Syndrome
by Margaret Kennedy and Mariya Apostolova
Neurol. Int. 2013, 5(2), e7; https://doi.org/10.4081/ni.2013.e7 - 25 Jun 2013
Cited by 11 | Viewed by 1
Abstract
Infectious mononucleosis is a clinical syndrome characterized by fever, lymphadenopathy and pharyngitis. Neurologic complications of infectious mononucleosis, such as the development of Guillain-Barre syndrome, have been rarely reported and usually present late in the course of the disease. We describe a case of [...] Read more.
Infectious mononucleosis is a clinical syndrome characterized by fever, lymphadenopathy and pharyngitis. Neurologic complications of infectious mononucleosis, such as the development of Guillain-Barre syndrome, have been rarely reported and usually present late in the course of the disease. We describe a case of a 29 year old male with no significant past medical history who was diagnosed with Guillain-Barre syndrome following an infection with Epstein-Barr virus associated infectious mononucleosis. Supportive treatment resulted in full recovery. Full article
636 KiB  
Case Report
The Coexistence of Multiple Sclerosis and Hereditary Spastic Paraparesis in a Patient
by Işıl Yazıcı, Nılufer Yıldırım and Yaşar Zorlu
Neurol. Int. 2013, 5(2), e6; https://doi.org/10.4081/ni.2013.e6 - 25 Jun 2013
Cited by 4 | Viewed by 1
Abstract
Multiple sclerosis (MS) is a chronic disease characterized by multiple areas of central nervous system inflammation, demyelination and axonal loss. Hereditary spastic paraparesis (HSP) is characterized clinically by progressive spasticity and weakness of the lower limbs and pathologically by retrograd axonal degeneration of [...] Read more.
Multiple sclerosis (MS) is a chronic disease characterized by multiple areas of central nervous system inflammation, demyelination and axonal loss. Hereditary spastic paraparesis (HSP) is characterized clinically by progressive spasticity and weakness of the lower limbs and pathologically by retrograd axonal degeneration of the corticospinal tracts and posterior columns. We identified a patient with clinical history and investigation findings consistent with the concurrence of both MS and HSP. Laboratory and radiological investigations, cognitive tests were performed. Genetic confirmation for spastin gene mutation has been completed. If this coexistence is not coincidence the mutation in the spastin gene may be a strong susceptibility locus for MS. Full article
721 KiB  
Article
Asymptomatic Hyper-Creatine-Kinase-Emia as Sole Manifestation of Inclusion Body Myositis
by Josef Finsterer, Claudia Stöllberger and Gabor G. Kovacs
Neurol. Int. 2013, 5(2), e11; https://doi.org/10.4081/ni.2013.e11 - 25 Jun 2013
Cited by 6 | Viewed by 1
Abstract
Sporadic inclusion body myositis (sIBM) usually manifests with painless weakness of the hand, finger and hip flexors. Absence of symptoms or signs, but mild hyper-CK-emia as the sole manifestation of IBM, has not been reported. We report the case of a 73-year-old male [...] Read more.
Sporadic inclusion body myositis (sIBM) usually manifests with painless weakness of the hand, finger and hip flexors. Absence of symptoms or signs, but mild hyper-CK-emia as the sole manifestation of IBM, has not been reported. We report the case of a 73-year-old male who presented with asymptomatic recurrent hyper-CK-emia ranging from 200 to 1324 U/L (n<171 U/L), since 10 years. Clinical neurologic investigation, nerve conduction studies and EMG were non-informative. Muscle biopsy surprisingly revealed sIBM. sIBM may be asymptomatic and may manifest with hyper-CK-emia exclusively. So, it has to be included in the differential diagnoses of asymptomatic hyper-CK-emia. Full article
622 KiB  
Article
Relation of Helicobacter pylori Infection and Multiple Sclerosis in Iranian Patients
by Nafiseh Mohebi, Mansoureh Mamarabadi and Mehdi Moghaddasi
Neurol. Int. 2013, 5(2), e10; https://doi.org/10.4081/ni.2013.e10 - 25 Jun 2013
Cited by 37 | Viewed by 1
Abstract
Multiple sclerosis (MS) is the most prevalent central nervous system demyelinating disease. There are known risk factors for MS. However, there is uncertainty in its protective factors. Few studies have demonstrated that some chronic infections may have protective effects on this disease. We [...] Read more.
Multiple sclerosis (MS) is the most prevalent central nervous system demyelinating disease. There are known risk factors for MS. However, there is uncertainty in its protective factors. Few studies have demonstrated that some chronic infections may have protective effects on this disease. We carried this study to investigate the relation between Helicobacter pylori (HP) infection and the prevalence and severity of MS. In this case-control study, 163 MS patients and 150 sex- and age- matched controls were included. Blood samples for IgG and IgM anti HP antibodies were collected from all individuals. Also, the Expanded Disability Status Scale (EDSS) was used to evaluate the MS patients. Suitable statistical analysis was applied. A Significant difference was observed in seropositivity between these two groups (P<0.001) but no significant difference was seen in seropositivity between conventional and opticospinal MS (P=0.522). No significant difference was observed in seropositivity among ages (P=0.075) and between genders (P=0.204). A significant difference was seen in EDSS value between seropositive and seronegative patients (P=0.017). We concluded that patients with HP infection had lower incidence of multiple sclerosis and MS patients with HP infection showed lower neurologic complications, which can demonstrate that HP infection may have a protective influence on MS pathogenesis. Full article
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