Abstract: Hypothermia is a rare and poorly understood complication of Multiple Sclerosis (MS). We report on a 66-year-old patient currently with Secondary Progressive MS (SP-MS) who developed unexplained hypothermia associated with multiple hospitalisations and we review the literature on this topic. In our case, magnetic resonance imaging (MRI) of the brain failed to highlight hypothalamic disease, but spinal MRI identified a number of spinal cord lesions. Given the incidence and clinical significance of spinal involvement in MS and the hypothermic disturbances observed in high Spinal Cord Injury (SCI), we hypothesise that upper spinal cord pathology, along with hypothalamic and brainstem dysfunctions, can contribute to hypothermia. PubDate: Wed, 21 Mar 2018 00:00:00 +000
Abstract: Central alveolar hypoventilation disorders denote conditions resulting from underlying neurologic disorders affecting the sensors, the central controller, or the integration of those signals leading to insufficient ventilation and reduction in partial pressures of oxygen. We report a patient who presented with a left lateral medullary ischemic stroke after aneurysm repair who subsequently developed a rare complication of CAH. Increased awareness of this condition’s clinical manifestations is crucial to make an accurate diagnosis and understand its complications and prognosis. PubDate: Wed, 21 Mar 2018 00:00:00 +000
Abstract: Episodic ataxia is a heterogenous group of uncommon neurological disorders characterised by recurrent episodes of vertigo, dysarthria, and ataxia for which a variety of different genetic variations have been implicated. Episodic ataxia type two (EA2) is the most common and also has the largest number of identified causative genetic variants. Treatment with acetazolamide is effective in improving symptoms, so accurate diagnosis is essential. However, a large proportion of patients with EA2 have negative genetic testing. We present a patient with a typical history of EA2 who had a novel variant in the CACNA1A gene not previously described. Report of such variations is important in learning more about the disease and improving diagnostic yield for the patient. PubDate: Sun, 11 Mar 2018 00:00:00 +000
Abstract: Background. Autonomic dysfunction is common in Multiple Sclerosis (MS) patients. Most spinal cord lesions entail some degree of autonomic nervous system dysfunction. MS patients may develop autonomic dysfunction later in their disease course. Methods. We report a patient with no prior history of MS presenting with orthostatic symptoms and diagnosed initially with postural orthostatic tachycardia syndrome (POTS). Four months later, she was diagnosed with radiologically isolated syndrome (RIS). The diagnosis was supported by imaging and CSF analysis. Conclusion. Our case sheds light on the need to consider autonomic dysfunction as an initial presentation of demyelinating pathology. Larger trials are needed to outline the possible association between POTS and RIS. PubDate: Sun, 25 Feb 2018 07:02:51 +000
Abstract: Cerebral toxoplasmosis is one of the most common causes of focal brain lesions in immunocompromised patients, such as those with human immunodeficiency virus (HIV). Differentiating toxoplasmosis from other central nervous system (CNS) lesions provides a significant clinical challenge. Magnetic resonance (MR) imaging of the brain is key to prompt diagnosis and treatment of cerebral toxoplasmosis. Several specific signs on MRI of brain have been described in recent literature including the “concentric target sign” and “eccentric target sign.” We report a case of successfully treated HIV-associated cerebral toxoplasmosis in which both MRI signs were present simultaneously. PubDate: Wed, 21 Feb 2018 00:00:00 +000
Abstract: Individuals with Down syndrome (DS) can develop Alzheimer’s disease as early as 30 to 40 years old, but parkinsonism is rarely described. We report on a 20-year-old woman with Down syndrome and parkinsonism who responded dramatically to carbidopa-levodopa. We propose that the occurrence of parkinsonism in individuals with DS may be underreported. Recognizing and treating this condition may improve quality of life. PubDate: Thu, 15 Feb 2018 00:00:00 +000
Abstract: Ischemic stroke occurring in patients with human immunodeficiency virus (HIV) needs to be approached with a vast differential diagnosis in mind. We report a case of middle-aged male patient with immune reconstituted HIV on therapy without known cardiovascular risk factors who had a right middle cerebral artery territory infarct. After a thorough evaluation, he received a final diagnosis of neurosyphilis-associated vasculitis leading to stroke. He recovered without any neurological deficits following treatment with intravenous benzylpenicillin. Neurosyphilis is an easily diagnosed and treatable cause of a stroke that can be an initial presentation of neurosyphilis but requires a high index of suspicion. PubDate: Tue, 13 Feb 2018 00:00:00 +000
Abstract: Idiopathic normal pressure hydrocephalus (iNPH) is the most common cause of hydrocephalus in adults. The diagnosis may be challenging, requiring collaborative efforts between different specialists. According to the International Society for Hydrocephalus and Cerebrospinal Fluid Disorders, iNPH should be considered in the differential of any unexplained gait failure with insidious onset. Recognizing iNPH can be even more difficult in the presence of comorbid neurologic disorders. Among these, idiopathic Parkinson’s disease (PD) is one of the major neurologic causes of gait dysfunction in the elderly. Both conditions have their peak prevalence between the 6th and the 7th decade. Importantly, postural instability and gait dysfunction are core clinical features in both iNPH and PD. Therefore, diagnosing iNPH where diagnostic criteria of PD have been met represents an additional clinical challenge. Here, we report a patient with parkinsonism initially consistent with PD who subsequently displayed rapidly progressive postural instability and gait dysfunction leading to the diagnosis of concomitant iNPH. In the following sections, we will review the clinical features of iNPH, as well as the overlapping and discriminating features when degenerative parkinsonism is in the differential diagnosis. Understanding and recognizing the potential for concomitant disease are critical when treating both conditions. PubDate: Sun, 21 Jan 2018 07:35:32 +000
Abstract: Inflammatory bowel disease is characterized by a chronic inflammatory state and is therefore associated with abnormalities in coagulation and a hypercoagulable state. Cerebral venous sinus thrombosis is a rare complication of inflammatory bowel disease yet contributes significant morbidity and mortality to those affected. Early diagnosis is critical, as a delay in diagnosis portends a worse prognosis. This paper seeks to highlight the increased risk of venous sinus thrombosis in patients with inflammatory bowel disease. We start by discussing the case of a seventeen-year-old female who presented with ulcerative colitis flare and developed new-onset seizures, found to be caused by a large venous sinus thrombosis. PubDate: Sun, 21 Jan 2018 00:00:00 +000
Abstract: We report an unusual clinical manifestation of ischemic stroke with acute right-sided asterixis affecting the arm as well as the leg due to a lesion in the left posterior limb of the internal capsula. After treatment with intravenous thrombolysis the patient made a good recovery. Notably, in this case unilateral asterixis affected the arm as well as the leg, resulting in postural and gait instability. In addition, damage in the basal ganglia-thalamo-cortical network, as in our patient, has to be distinguished from other supratentorial causes of acute asterixis like thalamic or frontal lobe lesions linked to the cerebello-brainstem-thalamo-frontal lobe circuits. PubDate: Thu, 18 Jan 2018 09:08:03 +000
Abstract: Chronic traumatic encephalopathy (CTE) is a neurodegenerative disease caused by head trauma. Diagnosis of this disease is difficult as reliable biomarkers have not been established and often this clinical entity is underappreciated with poor recognition of its clinical presentations (Lenihan and Jordan, 2015). The definitive diagnosis of CTE is determined by identification of neurofibrillary tangles in the perivascular space around the sulci in postmortem tissue (McKee et al., 2015). However, performing brain biopsies searching for neurofibrillary tangles is not a feasible option for early diagnosis. Thus, diagnosis of suspected CTE in the living has been based on clinical suspicion using proposed research criteria of clinical presentations. In addition, neuroimaging techniques have shown some promise in assisting diagnosis. Clinically, CTE is more commonly known to be associated with memory impairment and executive function disorder (Stern et al., 2013). However, here, we present two unique cases of prior professional football players where behavioral changes were the first identifying factors in clinical presentation and discuss possible neuroimaging options to help with CTE diagnosis. Because behavioral changes can be mistaken for other neuropsychological diseases, recognizing differing clinical constellations is critical to early diagnosis, early intervention, and improving patient care in suspected CTE. PubDate: Sun, 14 Jan 2018 07:55:49 +000
Abstract: Spontaneous spinal subdural hematoma (sSDH) is a rare condition outright. Moreover, cases that occur spontaneously in the absence of an identifiable etiology are considerably less common and remain poorly understood. Here, we present the case of a 43-year-old man with spontaneous sSDH presenting with acute onset low back pain and paraplegia. Urgent magnetic resonance imaging identified a dorsal SDH from T8 to T11 with compression of the spinal cord. Emergent T8–T10 laminectomies with intradural exploration and hematoma evacuation were performed. However, despite prompt identification and appropriate action, the patient’s recovery was modest and significant disability remained at discharge. This unique and unusual case demonstrates that spontaneous sSDH requires prompt surgical treatment to minimize associated morbidity and supports the association between the presence of severe neurological deficits upon initial presentation with less favorable outcomes. We performed a comprehensive systematic review of spontaneous sSDH of unknown etiology, which demonstrates that emergent surgical intervention is indicated for patients presenting with severe neurological deficits and the presence of these deficits is predictive of poor neurological outcome. Furthermore, conservative management should be considered in patients presenting with mild neurological deficits as spontaneous resolution followed by favorable neurological outcomes is often observed in these patients. PubDate: Tue, 26 Dec 2017 08:40:13 +000
Abstract: Herein, we report on a rare case of craniopharyngioma arising in the left temporal lobe with no prior history of head trauma or surgery. There was a solid-cystic mass in the left temporal lobe on MR images. To the best of our knowledge, this is the second case of a craniopharyngioma occurring in the temporal lobe. PubDate: Tue, 26 Dec 2017 00:00:00 +000
Abstract: Occurrence of cerebral ischemia in the posterior circulation as a result of severe internal carotid artery disease and persistent trigeminal artery is rare. An 81-year-old man with medical history of hypertension and ischemic stroke presented with dizziness, nausea, and mild dysarthria. Magnetic resonance imaging of the brain revealed acute infarcts in the left internal carotid artery territory. CT angiogram revealed a persistent trigeminal artery (PTA) and severe atherosclerosis. The patient developed new neurological symptoms and repeat imaging revealed new acute infarcts in the PTA distribution. After undergoing a left carotid endarterectomy with no complications, the patient was discharged to a skilled nursing facility with no recurrence of ischemic stroke. This case adds a rare complication of an infrequent vascular anomaly to the limited body of the literature. PubDate: Mon, 04 Dec 2017 08:36:57 +000
Abstract: Antidopaminergic drugs are known to induce extrapyramidal symptoms. Clebopride, a dopamine antagonist, also can produce parkinsonism, tardive dyskinesia, tardive dystonia, hemifacial dystonia, or oculogyric crisis; however, acute dystonic reaction caused by clebopride has not been reported in adults. We report two young men who experienced acute cervical dystonia within a few days of taking clebopride. The patients recovered after discontinuation of the drug. Physicians prescribing clebopride should be aware of the adverse effects of this drug. PubDate: Tue, 28 Nov 2017 00:00:00 +000
Abstract: Background. Continuous electroencephalography (CEEG) monitoring is used for detection of convulsive and nonconvulsive seizures and assessing the degree of encephalopathy in critically ill patients. A commonly seen encephalopathic pattern on CEEG is generalized periodic discharges with triphasic wave (TW) morphology. The underlying role and prognostic significance of TW in relationship to intracranial pressure (ICP) remain unknown. We present a case highlighting the relationship of TW with ICP in a case with severe traumatic brain injury (TBI). Method. Case report. Results. A patient with severe TBI and no underlying metabolic abnormalities was admitted to the neurocritical care unit. TW were seen on CEEG. The TW diminished during episodes of intracranial hypertension but reappeared with reduction of the intracranial pressure. Conclusion. This study highlights a possible favorable prognostic marker of finding TW in a patient with intracranial hypertension. We have proposed a preliminary understanding of the relationship between TW and intracranial hypertension, which may be helpful in formulating future studies involving larger cohorts. PubDate: Tue, 28 Nov 2017 00:00:00 +000
Abstract: Vitamin B12 deficiency results in hematological, neurological, and rarely dermatological complications. Subacute combined degeneration of the cord is one of the neurological complications, and usually the presenting symptom is paresthesia. Herein, we report a case of a 46-year-old man with subacute combined degeneration presenting with knuckle hyperpigmentation. PubDate: Wed, 22 Nov 2017 08:05:48 +000
Abstract: Peri-ictal magnetic resonance imaging (MRI) findings following seizure activity are a recognized phenomenon that is not well understood (Cole, 2004). Transient changes are not usually expected to be present in postictal MRI studies because of their rarity. Here, we present a unique case of peri-ictal MRI findings located in the occipital lobe, present in a 34-year-old female with recurrent occipital seizures occurring twice in four years. MRI changes completely resolved after both episodes with no residual focal damage. The peri-ictal occipital changes on MRI in this patient are unique because they have been captured on more than one occasion. Peri-ictal MRI findings are a known phenomenon with unknown pathophysiology, although attempts have been made to understand these findings. Though the MRI findings and presentation appear to be stroke-like or PRES-like, seizures should be kept in the differential for better treatment outcomes. PubDate: Tue, 07 Nov 2017 06:52:28 +000
Abstract: Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT) is a diagnostic conundrum as it may present with a myriad of nonspecific clinical features and laboratory and neuroimaging investigations are not diagnostic. We report a case of a 65-year-old female who presented with an acute febrile illness associated with headache and confusion, tangential thoughts, and loose association. Based on neutrophil leukocytosis in the full blood count and elevated inflammatory markers, she was commenced on empirical intravenous antibiotics suspecting meningoencephalitis. Further evaluation found a very high titer of both anti-thyroid peroxidase (anti-TPO) antibodies and anti-thyroid globulin antibodies. She was clinically and biochemically euthyroid. EEG showed right sided frontal intermittent rhythmic delta activity (FIRDA). Cranial MRI revealed age related cerebral atrophy and nonspecific periventricular white matter changes. A diagnosis of SREAT was made and she was treated with intravenous methylprednisolone followed by oral prednisolone. Her condition improved dramatically within 48 hours of starting steroids. SREAT is a diagnosis of exclusion in patients with a central nervous system disorder. There are no specific clinical features or investigative findings. Elevated anti-TPO antibodies are considered a hallmark of SREAT and steroid responsiveness supports the diagnosis. Prompt diagnosis and treatment reverses the neurological dysfunction in most cases. PubDate: Mon, 06 Nov 2017 06:33:32 +000
Abstract: Metastatic spinal cord compression (MSCC) is a medical emergency that requires rapid diagnosis and treatment to reduce pain, to preserve neurological functioning, and to prolong survival. The diagnosis of liver cancer is often neglected in the differential diagnosis of MSCC. Treatment is usually palliative and evolution is often fatal. This is a case report of a 28-year-old patient living in Mauritania hospitalized in November 2014 at the neurology department of Fann national teaching hospital in Dakar, for the management of a chronic dorsal spinal cord compression. The radiological and laboratory investigations done revealed the metastatic compression originating from a liver cancer with elevated alpha-fetoprotein and aspartate transaminase, positive hepatitis B surface antigen, and multiple metastasis in the lungs, mediastinum, ribs, iliac, and peritoneum. The hip joint X-ray showed a spontaneous fracture of the right femoral neck. The multidisciplinary treatment was palliative and the evolution was fatal within the month of hospitalization. Earlier diagnosis and treatment of MSCC may not have saved the life of the patient but may have prevented much suffering and would likely have prolonged the life of a young man. PubDate: Thu, 02 Nov 2017 00:00:00 +000
Abstract: Background. Stiff person syndrome (SPS) is a rare neurologic disorder characterized by muscle rigidity. It is a disorder of reduced GABA activity leading to increased muscle tone and often painful spasms. It generally presents in the axial musculature but rarely can involve only one limb, typically a lower extremity. In rare cases it can be paraneoplastic which generally resolves on treatment of the underlying neoplasm. Case Report. A 46-year-old male with a history of Hodgkin’s Lymphoma in remission presented with left upper extremity pain secondary to a diagnosis of Stiff Person Syndrome limited to his left upper extremity. He had previously benefitted from plasmapheresis and was on diazepam and baclofen at home with relatively good control of his symptoms. SPS had previously been diagnosed with EMG and anti-GAD-65 antibody titers and was confirmed by an elevated anti-GAD-65 antibody titer. He was treated with plasmapheresis and maximum doses of medical treatment including botulinum toxin with only transient mild improvement in his symptoms. Conclusion. This case represents a case of a rare disease that was refractory to all known therapies. It outlines the need for further understanding of this disorder in order to provide better symptomatic treatment or potentially more definitive care. PubDate: Wed, 25 Oct 2017 08:01:41 +000
Abstract: Unilateral hemichorea/hemiballism (HH) associated with contralateral neuroimaging abnormalities of the basal ganglia, which is characterized by T1 hyperintensity on magnetic resonance imaging (MRI) and is secondary to diabetic nonketotic hyperglycemia, is a rare and unique complication of poorly controlled diabetes mellitus (DM). Although almost all prior reports have documented rapid resolution of HH within days after normalization of blood glucose levels, medically refractory persistent HH has been noted. The experience of surgical intervention for persistent HH is limited. A 46-year-old, right-handed female patient with type 2 DM presented with refractory diabetic HH on the left side of 6 months’ duration despite DM control and neuroleptic medication usage. Image-guided deep brain stimulation (DBS) on the right globus pallidus internus (GPi) was performed. A mechanical micropallidotomy effect was observed and chronic stimulation of GPi was quite effective in symptomatic control of diabetic HH until a 16-month follow-up visit. DBS of the GPi can be an effective treatment for medically refractory diabetic HH. PubDate: Mon, 23 Oct 2017 07:01:45 +000
Abstract: Background. Common side effects of selective serotonin reuptake inhibitors (SSRIs) include tachycardia, drowsiness, tremor, nausea, and vomiting. Although SSRIs have less toxic side effects compared to more traditional antidepressants, serious and life threatening cases of SSRI overdose have been reported. We describe a 24-year-old multimorbid female who presented to the emergency department with rapid onset ascending sensorimotor paralysis, complicated by respiratory and cardiac arrest, found to have fatal levels of fluoxetine by toxicological analysis, not taken in a suicidal act. Results. Autopsy was performed at the Los Angeles County Medical Examiner’s Office of a female with no evidence of traumatic injury. Toxicological analysis revealed lethal levels of fluoxetine, toxic levels of diphenhydramine, and multiple other coingested substances at nontoxic levels. Neuropathological examination of the brain and spinal cord revealed no evidence of Guillain-Barre paralysis. Conclusions. Lethal levels of fluoxetine and multiple potential drug-to-drug interactions in our patient likely contributed to her unique signs and symptoms. This is the first case reporting neurologic signs and symptoms consisting of rapid onset ascending sensorimotor paralysis, hearing loss, respiratory failure, cardiac arrest, and death in a patient with lethal levels of fluoxetine. PubDate: Mon, 16 Oct 2017 00:00:00 +000
Abstract: Among other autonomic dysfunctions complicating acute spinal cord injury, deep hypothermia is rare but may induce serious cardiovascular complications. There are few pharmacological options to influence hypothermia. A 66-year-old woman was transferred to the intensive care unit (ICU) for serious cardiac arrhythmias (atrial fibrillation and asystole) in the context of a deep hypothermia (axillary temperature below 32°C). She had been admitted to the hospital two months before for an acute L4-L5 infectious spondylodiscitis without any initial neurological deficit. After surgery for epidural abscess drainage, she became paraplegic due to spinal cord infarction (from C7 to T6 levels) in the upper territory of the anterior spinal artery. In the ICU, the patient experienced several episodes of asystole and hypotension associated with a core body temperature below 35°C. Common causes of hypothermia (drugs, hypothyroidism, etc.) were excluded. A definitive pacemaker had to be inserted, but hypotension persisted. The prescription of oral progesterone (200 mg·d−1) helped to maintain a core temperature higher than 35°C, with a withdrawal of vasopressors. This case report illustrates that patients with incomplete spinal cord injury may present with delayed and deep hypothermia leading to serious cardiovascular complications. Progesterone could be able to influence positively central and peripheral thermal regulation. PubDate: Wed, 04 Oct 2017 00:00:00 +000
Abstract: A 54-year-old patient presented with mild right-sided weakness of hand and face with a National Institutes of Health Stroke Scale (NIHSS) of 2 and occlusion of the left middle cerebral artery (MCA) in the M1 segment with a large perfusion deficit on computed tomography (CT). Due to mild neurological deficits no IVT was performed. Nine hours after symptom onset the patient gradually deteriorated with a NIHSS fluctuating between 9 and 15. MRI showed a persistent occlusion of the MCA with a large diffusion-perfusion mismatch. Immediate endovascular thrombectomy was performed 14 hours after symptom onset with complete recanalization and complete clinical recovery. Although mechanical thrombectomy is generally considered an effective alternative strategy up to 8 hours after stroke onset, selected patients with a large diffusion/perfusion mismatch and small infarct cores may benefit from an expanded therapeutic window. PubDate: Wed, 04 Oct 2017 00:00:00 +000
Abstract: Multiple sclerosis (MS) is a chronic inflammatory demyelinating disease of the central nervous system. The etiology is insufficiently understood. Autoimmune, genetic, viral, and environmental factors have been hypothesized. MS is twice as common in women as in men between the ages of 20 and 50 years. There is no known cure for MS. Current medical treatment helps to prevent new attacks and improve function after an attack. MS is diagnosed by physical examination, diagnostic imaging, and examination of cerebral spinal fluid. The most common physical signs and symptoms of MS include constitutional symptoms, muscle weakness, motor and autonomic spinal cord symptoms, paresthesias, and vision changes. Here we present a case of MS diagnosed in a 33-year-old male with facial myokymia of left eyelid, which progressed to left hemifacial spasm. This is an unusual presentation for multiple sclerosis. An awareness of this presentation not only may lead to an earlier diagnosis in some patients but can be a sign of relapse in patients with established multiple sclerosis. PubDate: Sun, 17 Sep 2017 10:32:07 +000
Abstract: Intraspinal synovial cyst is a rare cause of myelopathy. These cysts present most often in the lumbar and cervical parts of the spine but are more infrequent in the thoracic spine. We present a case of a 73-year-old man with an intraspinal, extradural synovial cyst at the Th2-3 level causing paraesthesia and weakness in the legs. A laminectomy and excision of the cyst were performed and the patient recovered fully. In the thoracic spine, synovial cysts are almost exclusively found in the lower part. Laminectomy, with excision, is the treatment of choice, although steroid injections have been described. PubDate: Thu, 07 Sep 2017 00:00:00 +000
Abstract: Background. Porencephaly is an extremely rare neurological disease characterized by the presence of solitary or multiple degenerative cerebrospinal fluid (CSF) cavities within the brain parenchyma. Case Report. We describe a case involving a 23-year-old male who presented with involuntary movements of the left upper limb of 6 months’ duration. A diagnosis of porencephaly was confirmed by magnetic resonance imaging (MRI). Conclusion. The rarity of occurrence and atypical presentation of such a lesion present a challenge to clinicians. Little is known about the pathogenesis and appropriate management of porencephaly. Further studies of the implications of porencephaly for neurodevelopment and behavior are needed. PubDate: Tue, 05 Sep 2017 00:00:00 +000
Abstract: Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease caused by the JC virus, a polyomavirus that can be reactivated under certain immunosuppressive conditions, such as AIDS, immunomodulatory therapy, and haematological malignancies. However, a few cases of immunocompetent patients have been reported in which no immunodeficiency was present. We describe the case of an 83-year-old immunocompetent man who presented with severe cerebellar symptoms with an MRI scan suggestive of severe demyelinating disease. We were not able to identify any occult immunosuppression or malignancy in our patient. PubDate: Thu, 24 Aug 2017 10:03:06 +000
Abstract: The systemic effects of digoxin toxicity have been well-known. However, there has been no case citing the effects of intrathecal digoxin in light of end-stage renal disease in the elderly. Here, we report on the case of the successful management of accidental intrathecal digoxin administration in an elderly male with end-stage renal disease. PubDate: Tue, 22 Aug 2017 07:43:35 +000
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