Fulminant subacute sclerosing panencephalitis (SSPE) presented with acute hemiparesis in a 13-year-old girl with perinatally acquired HIV infection

A 13-year-old girl with perinatally acquired HIV infection was admitted to us with acute onset, right-sided hemiparesis of 30 days duration and right-sided myoclonic jerks of 2 days duration affecting the face, upper and lower limbs. On examination, she exhibited increased tone and a pyramidal pattern of weakness in her right upper and lower limbs, along with spontaneous multifocal myoclonic jerks in the affected area. IgG levels in the serum and cerebrospinal fluid for measles were significantly elevated. Brain MRI depicted T2-weighted-hyperintensities in the subcortical white matter. The electroencephalogram demonstrated evidence of lateralised long interval periodic discharges. This patient had no past behavioural problems or poor academic performance. This case underlines the fact that, though subacute sclerosing panencephalitis (SSPE) is a chronic disease, a rare fulminant form of SSPE might develop acutely and atypically, with an increased proclivity for HIV-infected patients.

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A Case of Classic Raymond Syndrome

Case Reports in Neurological Medicine ◽

10.1155/2012/583123 ◽

2012 ◽

Vol 2012 ◽

pp. 1-3

Author(s):

Nicholas George Zaorsky ◽

Jin Jun Luo

Keyword(s):

Nerve Palsy ◽

Brain Mri ◽

Abducens Nerve ◽

Acute Onset ◽

Common Type ◽

Facial Paresis ◽

Clinical Observations ◽

The Face ◽

The Common ◽

The Right

Classic Raymond syndrome consists of ipsilateral abducens impairment, contralateral central facial paresis, and contralateral hemiparesis. However, subsequent clinical observations argued on the presentation of facial involvement. To validate this entity, we present a case of classic Raymond syndrome with contralateral facial paresis. A 50 year-old man experienced acute onset of horizontal diplopia, left mouth drooling and left-sided weakness. Neurological examination showed he had right abducens nerve palsy, left-sided paresis of the lower part of the face and limbs, and left hyperreflexia. A brain MRI showed a subacute infarct in the right mid-pons. The findings were consistent with those of classic Raymond syndrome. To date, only a few cases of Raymond syndrome, commonly without facial involvement, have been reported. Our case is a validation of classic Raymond syndrome with contralateral facial paresis. We propose the concept of two types of Raymond syndrome: (1) the classic type, which may be produced by a lesion in the mid-pons involving the ipsilateral abducens fascicle and undecussated corticofacial and corticospinal fibers; and (2) the common type, which may be produced by a lesion involving the ipsilateral abducens fascicle and undecussated corticospinal fibers but sparing the corticofacial fibers.

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Acute-onset paraplegia as an unexpected complication under general anesthesia in supine position during abdominal endovascular aneurysm repair: a case report

JA Clinical Reports ◽

10.1186/s40981-021-00447-7 ◽

2021 ◽

Vol 7 (1) ◽

Author(s):

Atsushi Morio ◽

Hirotsugu Miyoshi ◽

Noboru Saeki ◽

Yukari Toyota ◽

Yasuo M. Tsutsumi

Keyword(s):

Spinal Cord ◽

General Anesthesia ◽

Supine Position ◽

Spinal Cord Ischemia ◽

Endovascular Aneurysm Repair ◽

Acute Onset ◽

Lower Limbs ◽

Canal Stenosis ◽

Cord Injury ◽

Aneurysm Repair

Abstract Background Acute onset paraplegia after endovascular aneurysm repair (EVAR) is a rare but well-known complication. We here show a 79-year-old woman with paraplegia caused by static and dynamic spinal cord insult not by ischemia after EVAR. Case presentation The patient underwent EVAR for abdominal aortic aneurism under general anesthesia in the supine position. She had a medical history of lumbar canal stenosis. After the surgery, we recognized severe paraplegia and sensory disorder of lower limbs. Although the possibility of spinal cord ischemia was considered at that time, postoperative magnetic resonance imaging (MRI) revealed burst fracture of vertebra and compressed spinal cord. Conclusions Patients with spinal canal stenosis can cause extrinsic spinal cord injury even with weak external forces. Thus, even after EVAR, it is important to consider extrinsic factors as the cause of paraplegia.

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The Importance of Meanings of Antiretroviral Treatment and Care Providers for Adherence and Transitioning to Adult Services Among Youth With Perinatally Acquired HIV Infection

Journal of HIV/AIDS & Social Services ◽

10.1080/15381501.2014.912172 ◽

2015 ◽

Vol 14 (3) ◽

pp. 257-276 ◽

Cited By ~ 2

Author(s):

Mylène Fernet ◽

Normand Lapointe ◽

Joseph J. Lévy ◽

Johanne Samson ◽

Joanne Otis ◽

...

Keyword(s):

Hiv Infection ◽

Antiretroviral Treatment ◽

Care Providers ◽

Adult Services ◽

Perinatally Acquired

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Highly Active Anti-Retroviral Treatment (HAART) Medication Adherence in Adolescents with Perinatally-Acquired HIV Infection

Journal of Allergy and Clinical Immunology ◽

10.1016/j.jaci.2009.12.306 ◽

2010 ◽

Vol 125 (2) ◽

pp. AB78

Author(s):

R.J. Sporter ◽

E.A. Meyer ◽

D.W. Rosenthal

Keyword(s):

Medication Adherence ◽

Hiv Infection ◽

Highly Active ◽

Perinatally Acquired

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Haemiballism/haemichorea: an atypical presentation of ischaemic stroke

BMJ Case Reports ◽

10.1136/bcr-2020-240439 ◽

2021 ◽

Vol 14 (6) ◽

pp. e240439

Author(s):

Raman Nohria ◽

Stacey Bennett ◽

Yasmin Ali O'Keefe

Keyword(s):

Brain Mri ◽

Acute Onset ◽

Cerebral Peduncle ◽

Atypical Presentation ◽

Cerebrospinal Fluid Analysis ◽

Medication Therapy ◽

Fluid Analysis ◽

Ct Head ◽

The Right ◽

Distressing Condition

A 76-year-old man was admitted to the hospital with acute onset of involuntary movements of the left side of his body. His neurological examination revealed he was oriented only to himself, and aforementioned movements of his left arm and leg. CT head demonstrated old infarcts in his right aspect of his pons and basal ganglia. Cerebrospinal fluid analysis was unremarkable. He initially had a normal blood glucose with an elevated anion gap and elevated creatine kinase. Brain MRI showed a small lacunar-type ischaemic infarct within the anteromedial aspect of the right cerebral peduncle, which localised to his haemiballism. To prevent worsening rhabdomyolysis associated with his haemiballism, the primary team initiated both tetrabenazine and diazepam. His movements improved after 1 week of medication therapy. This report discusses a thorough workup for this movement disorder and when to intervene for this distressing condition.

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Impact of Disclosure over Time on the Emotional Well-Being of Children with Perinatally Acquired HIV Infection in South Africa

Journal of Developmental & Behavioral Pediatrics ◽

10.1097/dbp.0000000000001008 ◽

2021 ◽

Vol Publish Ahead of Print ◽

Author(s):

Janice Buckley ◽

Kennedy Otwombe ◽

Celeste Joyce ◽

Given Leshabane ◽

Lisa Galvin ◽

...

Keyword(s):

South Africa ◽

Hiv Infection ◽

Well Being ◽

Perinatally Acquired ◽

Over Time

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Infantile Neurodegeneration and Hair Changes: A Rare Case of Menkes Disease

Dubai Medical Journal ◽

10.1159/000521155 ◽

2022 ◽

pp. 1-4

Author(s):

Nikhil Vikas Pawar ◽

Fatima Farid Mir

Keyword(s):

Seizure Activity ◽

Multidisciplinary Care ◽

Menkes Disease ◽

Symptomatic Treatment ◽

Serum Copper ◽

Subcortical White Matter ◽

Acute Onset ◽

Hair Shaft ◽

Recurrent Seizure ◽

Antiepileptic Medications

A 4-month-old, previously healthy boy presented with acute onset of prolonged, recurrent seizure activity followed by neurodevelopmental deterioration and concurrent hair shaft hypopigmentation with fragility. Initial evaluation revealed significant low serum copper and ceruloplasmin, electrical status epilepticus on electroencephalography, and generalized subcortical white matter changes with diffuse tortuosity of intracranial vessels on MRI brain. In addition, a genetic study with whole-genome sequencing demonstrated a hemizygous pathogenic variant at c.2179G>A p(Gly727Arg) on ATP7A, thereby confirming the diagnosis of Menkes disease. Symptomatic treatment with antiepileptic medications was provided along with an urgent referral to an advanced center for multidisciplinary care and copper histidine replacement therapy.

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Bilateral symmetrical deep gray matter involvement and leptomeningeal enhancement in a child with MOG-IgG-associated encephalomyelitis

BMC Neurology ◽

10.1186/s12883-020-02041-3 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Weibing Shen ◽

Yaner Zhang ◽

Chenguang Zhou ◽

Yaoyao Shen

Keyword(s):

Gray Matter ◽

Mononuclear Cells ◽

Demyelinating Disease ◽

Brain Mri ◽

Acute Onset ◽

Neck Stiffness ◽

Leptomeningeal Enhancement ◽

Barr Virus ◽

Disturbance Of Consciousness ◽

Deep Gray Matter

Abstract Background Currently, myelin oligodendrocyte glycoprotein (MOG)-IgG-associated encephalomyelitis (MOG-EM) is regarded as an independent inflammatory demyelinating disease. Magnetic resonance imaging (MRI) abnormalities occur in 44.4% of patients with MOG-EM. However, symmetrical deep gray matter involvement with leptomeningeal enhancement is rarely described in the literature. Case presentation A 3-year-old boy was admitted to our hospital because of acute onset fever, headache, vomiting and disturbance of consciousness. Neurological examination showed somnolence, neck stiffness and positive Kernig’s sign. Brain MRI demonstrated bilateral symmetrical lesions in the basal ganglia and thalamus as well as diffuse leptomeningeal enhancement along the sulci of bilateral hemisphere. Cerebrospinal fluid analysis demonstrated increased cell count (7 cells/mm3, mononuclear cells dominant) and protein (1.17 g/L) without glucose and chloride abnormality. Work-up for infectious and autoimmune causes, serum MOG IgG was positive by cell based assay. Therefore, a diagnosis of MOG-EM was established according to the international recommendatory criteria in 2018. He was administrated with intravenous methylprednisolone followed by oral corticosteroids and had recovered completely within 1 week. Conclusions In the setting of meningoencephalitis-like clinical presentation with bilateral symmetrical deep gray matter involvement, MOG-EM should be distinguished from other infectious and autoimmune disorders, such as Epstein-Barr virus (EBV) encephalitis, Japanese encephalitis and Anti-NMDA receptor (NMDAR) encephalitis. Besides, aseptic meningitis associated with leptomeningeal enhancement may be an atypical phenotype of MOG-EM.

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