Neurocognitive recovery in SMART syndrome: A case report

Cephalalgia ◽  
2010 ◽  
Vol 31 (3) ◽  
pp. 372-376 ◽  
Author(s):  
Jennifer Bradshaw ◽  
Luke Chen ◽  
Michael Saling ◽  
Greg Fitt ◽  
Andrew Hughes ◽  
...  

Introduction: Stroke-like migraine attacks after radiation therapy, or SMART syndrome, is characterised by migraine-like headache with or without aura, transient neurological dysfunction, including seizures, and gyriform enhancement on magnetic resonance imaging (MRI) which resolves over a period of weeks. Detailed neuropsychological characterisation in SMART syndrome is lacking and there are no published data on the course and pattern of neurocognitive recovery. Results: The acute clinical presentation was one of unstable, fluctuating neurocognitive disturbances, complicated by seizure activity, followed by a longer term lag in the recovery of focal neuropsychological deficits which, we believe, was due to the more slowly resolving cerebral effects of SMART. Conclusions: To our knowledge, this is the first case of SMART syndrome in which neuropsychological functioning has been comprehensively and serially examined. This case is also unique due to the development of complex partial seizures. We suggest that epileptiform activity during clinical seizures should not be regarded as inconsistent with a diagnosis of SMART, provided that the seizures do not explain the onset of the other clinical and radiological features.

PEDIATRICS ◽  
1985 ◽  
Vol 76 (4) ◽  
pp. 612-613
Author(s):  
ROBERT C. WOODY

The increasing availability of videorecording cameras and cassette recorders now permits the visual documentation of medical events in children at home by parents. On two occasions recently, we asked families to videorecord their children's presumed seizure activity at home. In the first case, a 10-month-old white boy had frequent "spells" which by history appeared to be complex partial seizures. Routine awake and asleep EEG tracings were normal, and the family resisted hospital admission for financial reasons. Anticonvulsant medications were prescribed, and the family suggested that they borrow their parent's videocassette recorder to document their son's spells at home. Their videorecordings produced a high quality, permanent record of definite complex partial symptom activity clearly revealing eye deviation, nystagmus, and associated head and arm tonic activity.


2021 ◽  
Vol 11 (01) ◽  
pp. e262-e264
Author(s):  
Matthias Lange ◽  
Bernd Mitzlaff ◽  
Florian Beske ◽  
Holger Koester ◽  
Wiebke Aumann ◽  
...  

AbstractCentral nervous system (CNS) tumors are the most common solid tumors in children and adolescents. However, in neonates and children aged younger than a year, they are very rare. Clinical presentation in neonates is often subtle and nonspecific. When neurological symptoms are apparent at this age, cranial ultrasound (CUS) is often done as the initial evaluation, with a standard approach through the anterior fontanel (AF), followed by further imaging, such as magnetic resonance imaging (MRI), if necessary. We report the first neonatal case of a rapidly progressive diffuse midline glioma positive for histone H3 K27M mutation (World Health Organization [WHO] grade IV) in which using extended (transmastoid) CUS studies through the mastoid fontanelle (MF) in the second month of life defined the lesion in the brainstem.


2021 ◽  
pp. 66-68
Author(s):  
Kiran Shah ◽  
Ishant Bhanarkar ◽  
Vasavdatta Sharma ◽  
Durvesh Bhangale ◽  
Vinod Pawara ◽  
...  

Acromegaly is a condition of excessive somatic growth and distorted proportion due to hypersecretion of growth hormone(GH) and insulin-like growth factor 1 (IGF-1). Insidious clinical manifestation of GH excess as a result of GH-secreting pituitary adenoma renders acromegaly a disease with typically delayed diagnosis. We report a 29-year old male planned to undergo reconstruction of the nose. The patient was referred for medical tness. Given the clinical features consistent with acromegaly the patient was evaluated further for diagnosis. Laboratory investigation showed raised IGF-1and non-suppressed GH post 75gms glucose, amid normal cortisol, corticotropin(ACTH), prolactin, testosterone, and thyroid function tests. He did not have dysglycemia. Magnetic resonance imaging (MRI) of the pituitary revealed a pituitary macroadenoma consistent with acromegaly. This case highlights the notable absence of recognizing the clinical presentation of acromegaly in this patient by his medical care physician and surgeons, and therefore the importance of thorough history taking, attention, and observation in making a new diagnosis that has the potential to alter a patient's health care and alleviate impending complications, morbidity and/or mortality


Author(s):  
Myriam Irislimane ◽  
François Guilbert ◽  
Jean-Maxime Leroux ◽  
Lionel Carmant ◽  
Dang Khoa Nguyen

A 52-year-old woman was referred for a progressive neurological condition which started a year before with continuous irregular twitching of the right facial and arm muscles as well as the tongue and palate, followed by progressive dysphagia, right hemiparesis, ataxia, dysphasia and dysarthria. Though magnetic resonance imaging (MRI) at clinical presentation and a year after were both normal (Figure A1), Rasmussen's encephalitis (RE) was strongly suspected and treatment with corticosteroids and immunoglobulins were begun. A third MRI, two years after onset, revealed mild T2 hyperintense subcortical white matter changes over the left perisylvian region (Figure A) which confirmed our initial suspicion of RE as she now met clinical, electrophysiological and morphological criterias for RE. While waiting for a cerebral biopsy after unsatisfactory response from antiepileptic drugs, corticosteroids and immunoglobulins, her condition markedly worsened less than three years after onset with the sudden occurrence of status epilepticus requiring intubation and continuous infusions of midazolam and propofol.


2007 ◽  
Vol 65 (3b) ◽  
pp. 841-844 ◽  
Author(s):  
Henrique Barbosa Ribeiro ◽  
Tadeu Ferreira de Paiva Jr ◽  
Gustavo Pignatari Rosas Mamprin ◽  
Milton Luiz Gorzoni ◽  
Antônio José da Rocha ◽  
...  

Carcinomatous encephalitis is a rare entity, originally described by Madow and Alpers in 1951, which is characterized by tumoral spreading perivascular, without mass effect. Clinical manifestations such as hemiparesis, seizures, ataxia, speech difficulties, cerebrospinal fluid findings as well as computed tomography are nonspecific. This leads the physician to pursue more frequent diseases that could explain those manifestations - toxic, metabolic, and/or infectious encephalopathy. A magnetic resonance imaging (MRI) with gadolinium, the method of choice, presumes the diagnosis. Previous reports of this unusual form of metastatic disease have described patients with prior diagnosis of pulmonary adenocarcinoma. We present the case of carcinomatous encephalitis in a 76-years-old woman as the primary manifestation of occult pulmonary adenocarcinoma with its clinical, imaging, and anatomopathological findings.


2016 ◽  
Vol 8 (2) ◽  
pp. 87-92
Author(s):  
Lucas M. Gutierrez ◽  
Matias Cullari ◽  
Maximiliano Ranaletta ◽  
Ignacio Tanoira ◽  
César Gomez ◽  
...  

Introducción: El síndrome del espacio cuadrilátero (QSS) es un cuadro clínico causado por la compresión nerviosa y/o vascular a nivel del espacio axilar lateral. La prevalencia de este síndrome se desconoce y se cree que es baja, sin embargo, formas de presentación incompleta podrían llevar a que el mismo sea subdiagnosticado. Objetivo: Describir un protocolo diagnóstico utilizado en una serie de casos consecutivos de pacientes con omalgia inespecífica y sospecha de QSS. Materiales y Métodos: De manera prospectiva se evaluaron 10 pacientes con omalgia agravada con la rotación externa forzada, uno de ellos con síntomas bilaterales. Se realizó electromiografía (EMG) de deltoides y redondo menor bilaterales, con estudios de conducción nerviosa. Los valores se compararon con el hombro sano, y con un grupo control. El diagnóstico se confirmó mediante resonancia magnética nuclear (RMN). Resultados: El EMG mostró en todos los casos atrapamiento con compromiso axonal de la rama para el redondo menor, con preservación de la inervación del deltoides. La RMN mostró en los 11 hombros afectados atrofia selectiva del redondo menor. Conclusión: El QSS en sus formas incompletas suele ser subdiagnosticado, siendo necesario mantener un alto índice de sospecha clínica frente a un cuadro de omalgia inespecífica. Palabras Claves: espacio cuadrilátero, omalgia, electromiografía, redondo menor, nervio axilar. Introduction: The quadrilateral space syndrome (QSS) has clinical features caused by a nervous and /or vascular compression at the lateral axillary space. The prevalence of this syndrome is unknown but it is believed to be low. Nevertheless, some cases with an incomplete clinical presentation might be misdiagnosed. Purpose: To describe a diagnostic protocol used in a series of consecutive cases of patients with non-specific omalgia and suspected QSS. Materials and Methods: Ten patients of both genders suffering shoulder pain aggravated by forced external rotation were prospectively evaluated.  One of them had bilateral symptoms. Evaluation included clinical examination, electromyography (EMG) and nerve conduction studies of the deltoid and teres minor bilaterally. The results were compared with an age-matched control group. Diagnosis was confirmed by magnetic resonance imaging (MRI). Results: The EMG showed in all the cases a selective entrapment of the branch for the teres minor, with preservation of the innervation of the deltoid. The RMN showed in the 11 affected shoulders selective atrophy of the teres minor. Conclusion: Incomplete presentations of the QSS are usually underdiagnosed, making it necessary to maintain a high index of clinical suspicion in front of any non-specific omalgia case. Key words: quadrilateral space, omalgia, electromyography, teres minor, axillary nerve.


2016 ◽  
Vol 2016 ◽  
pp. 1-3
Author(s):  
Güner Koyuncu Çelik ◽  
Erkan Yildirim

A 79-year-old woman was admitted to our emergency department with complaints of fainting and loss of consciousness three times during the past month. She was diagnosed with epilepsy and started to be treated with antiepileptic drug. Physical examination showed, in the left eye, chemosis, limited eye movements in all directions, and minimal exophthalmos as unexisting symptoms on admission developed on the sixth day. Orbital magnetic resonance imaging (MRI) and digital subtraction angiography (DSA) imaging revealed a carotid cavernous fistula (CCF). Epileptic attacks and ophthalmic findings previously present but diagnosed during our examinations were determined to ameliorate completely after performing the coil embolization. Based on literature, we present the first case with nontraumatic CCF manifesting with epileptic seizures and intermittent eye symptoms in the present report.


2017 ◽  
Vol 08 (02) ◽  
pp. 277-280 ◽  
Author(s):  
Kamble Jayaprakash Harsha ◽  
Jeevan S. Nair

ABSTRACTTurner syndrome (TS) is a rare genetic disease due to the absence of one X chromosome. Patients with TS have more subtle neurological/neuropsychiatric problems, while headache is an uncommon clinical presentation which needs attention. We report a 12-year-old child presenting with typical cough headache. Her magnetic resonance imaging revealed Chiari I malformation associated with TS. To the best of our knowledge, Chiari I malformation associated with TS is not described in literature. We report the first case of TS associated with Chiari I malformation. Interestingly, Chiari I malformation is also associated with Noonan’s syndrome, which is a close morphological mimicker of TS, raising the possibility of sharing similar pathogenesis in both conditions.


2016 ◽  
Vol 23 (3) ◽  
pp. 483-486 ◽  
Author(s):  
Kalliopi Pitarokoili ◽  
Kerstin Hellwig ◽  
Carsten Lukas ◽  
Ralf Gold

We report the case of a post-progressive multifocal leukoencephalopathy (PML), multiple sclerosis (MS) patient with an uncomplicated pregnancy and delivery. A 28-year-old woman on natalizumab (total of 49 infusions) was diagnosed with PML due to typical magnetic resonance imaging (MRI) and clinical presentation. John Cunningham virus (JCV) was detected in the cerebrospinal fluid (CSF) during the immune reconstitution inflammatory syndrome (IRIS). Nine months after PML onset, JCV negativity in the CSF was observed. MS was stabilised with dimethyl fumarate (DMF), and 18 months later, a desired pregnancy was reported, resulting in the birth of a healthy boy. Our report gives new hope regarding family planning for post-PML patients.


2012 ◽  
Vol 2012 ◽  
pp. 1-3
Author(s):  
Alberto Peña-Valenzuela ◽  
Nathalia García León

Spindle cell lipoma of the hypopharynx is an extremely rare entity. Here, we present the first case of this lesion originated in the cricopharyngeal region, with symptoms of chronic progressive dysphagia, which can be confused with other pathologies; endoscopic and magnetic resonance imaging (MRI) evaluation are the methods of choice for its diagnostic approach. The best therapeutic approach is endoscopic resection with rapid recovery and few complications. Long-term followup is recommended, either endoscopic or imaging, given that it can be confused with an undiagnosed liposarcoma; additionally, its long-term behavior is unknown.


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