Movement Disorders Resulting From Bilateral Basal Ganglia Lesions in End-Stage Kidney Disease: A Systematic Review.

OBJECTIVE: The basal ganglia (BG) are susceptible to fluctuations in blood urea levels, sometimes resulting in movement disorders. We described patients with end-stage kidney disease (ESKD) presenting with movement disorders associated with bilateral BG lesions on imaging. METHODS: We report four patients and systematically reviewed all published cases of ESKD presenting with movement disorders and bilateral BG lesions (EBSCOhost and Ovid). RESULTS: Of the 72 patients identified, 55 (76.4%) were on regular dialysis. Parkinsonism was the most common movement disorder (n = 39; 54.2%), followed by chorea (n = 24; 33.3%). Diabetes mellitus (n = 51; 70.8%) and hypertension (n = 16; 22.2%) were the most common risk factors. Forty-three (59.7%) were of Asian ethnicity. Complete clinical resolution was reported in 17 (30.9%) patients, while 38 (69.1%) had incomplete clinical resolution with relapse. Complete radiological resolution occurred in 14 (34.1%) patients. CONCLUSION: Movement disorders associated with BG lesions should be recognized as a rare and potentially reversible metabolic movement disorder in patients with ESKD.

Labrune's Syndrome Presenting With Stereotypy-Like Movements and Psychosis: A Case Report and Review.

Labrune's syndrome, or leukoencephalopathy with brain calcifications and cysts (LCC), is a rare genetic syndrome with variable neurological presentations. Psychiatric manifestations and involuntary movements are uncommonly reported. We report the case of a 19-year-old female, initially diagnosed with Fahr's syndrome, who presented to us with acute psychosis, abnormal behavior and involuntary movements. Her brain computed tomography showed extensive bilateral intracranial calcifications without cysts. Genetic testing detected two compound heterozygous variants, NR_033294.1 n.*9C>T and n.24C>T, in the SNORD118 gene, confirming the diagnosis of LCC. We discuss the expanding phenotypic spectrum of LCC and provide a literature review on the current diagnosis and management of this rare syndrome.

Heterotopic ossification in psammomatous spinal meningioma: a diagnostic controversy.

Heterotopic ossification (HO), a synonym for osseous metaplasia, is a pathological phenomenon, characterized by abnormal bone formation outside the skeletal system observed commonly in various neoplastic and non-neoplastic diseases. HO occurring in meningioma is exceptionally rare. We reportherein an unusual case of spinal meningioma containing numerous calcified psammoma bodies and extensive HO in a 75-year-old woman, who presented with progressive worsening bilateral lower limb weakness and numbness. The presence of remarkable bone formation within a meningioma is controversial among pathologists; while some regard them as psammomatous meningioma as the primary diagnosis, others prefer osteoblastic meningioma, a form of metaplastic meningioma. There is compelling molecular data to advocate that HO is an active disease process involving metaplastic (osseous) differentiation of meningioma stroma mesenchymal stem-like cells, but not the meningothelial-derived tumor cells. Henceforth, the term "metaplastic meningioma" may not be appropriate in this context. A plausible designation as "psammomatous meningioma with osseous metaplasia" defines this entity more accurately. This paper highlights the need for a unifying nomenclature to reduce diagnostic controversy caused by conflicting terms in the literature. The possible pathogenesis of this intriguing phenomenon is discussed.

Bickerstaff brainstem encephalitis with Guillain-Barré syndrome overlap following chlamydia infection.

Bickerstaff brainstem encephalitis (BBE) is a rare autoimmune encephalitis characterised by ataxia, ophthalmoplegia and altered consciousness. An overlap between BBE with Guillain-Barré syndrome (GBS) shows similar clinical and immunological features. We report a case of BBE with GBS overlap secondary to Chlamydia pneumoniae infection. The triad of altered consciousness, ataxia and ophthalmoplegia were present in the patient. The investigations included cerebrospinal fluid cytoalbuminological dissociation, nerve conduction test that showed prolonged or absent F wave latencies, hyperintensity in the left occipital region on brain MRI and diffuse slow activity on the electroencephalogram. The chlamydia serology was positive indicating a postinfectious cause of BBE syndrome. He required artificial ventilation as his consciousness level deteriorated with tetraparesis, oropharyngeal and respiratory muscle weakness. Immunotherapy with intravenous immunoglobulin and methylprednisolone was commenced. He made good recovery with the treatment. Prompt recognition of this rare condition following chlamydia infection is important to guide the management.

Low lying larynx.

We report a rare case of laryngotracheal anomaly and its possible etiology and mode of presentation. A teenager presented with voice change and a neck lump. Investigations revealed a laryngeal anomaly in which the larynx was hyperdescended. It was accompanied by low lying thyroid gland and hyoid bone together with an absence of a cervical segment of the esophagus and trachea. The anomaly only became noticeable secondary to pubertal changes in the thyroid cartilage of the teenager. An embryological defect during the formation of the laryngotracheal tube and esophagus is a possible explanation of this anomaly. The present case probably represents the third reported of its kind.

Mother with post-partum group B Streptococcus meningitis and cerebellar abscess.

We report the case of a 25-year-old Malay woman, admitted for preterm delivery at 35 weeks' gestation. Vaginal swab did not isolate any organism. She delivered a baby girl who developed respiratory distress syndrome, requiring ventilation. Although chest radiograph showed hyaline membrane disease with pneumonia, septic workout was negative. The mother was discharged on the next day. Seven days postpartum, the mother presented with fever and fits and was diagnosed to have meningo-encephalitis. Lumbar puncture isolated group B Streptococcus (GBS) and MRI revealed a superior cerebellar abscess. She was treated and survived the episode. This case illustrates the uncommon situation where GBS infection was confirmed via maternal septic workout rather than neonatal, although both presented with severe disease.