Rapid-Onset Weakness and Numbness in a Patient With Systemic Lupus Erythematosus

A 33-year-old woman with systemic lupus erythematosus, diagnosed 2 years prior and treated with hydroxychloroquine, sought care for a 4-week history of pain and paresthesias in her low back and lower extremities. She described a bandlike sensation of numbness starting in her midback which descended to both legs. Her symptoms progressed to constipation and inability to urinate adequately. She reported difficulty with ambulation. Over the course of 1 week of hospitalization, urinary and fecal incontinence developed. On examination, she was alert and appropriately oriented. She had a malar rash and swelling of the metacarpophalangeal joints consistent with bilateral hand synovitis. Neurologic examination indicated hyperreflexia with brisk patellar and Achilles tendon reflexes bilaterally. She had trace motor weakness of the hip flexors, quadriceps, and hamstrings. She had loss of pinprick and temperature sensation in the lower extremities, extending beyond the saddle area to the T12 dermatome. Vibration perception and proprioception were preserved. She had a positive Babinski sign in the left foot. Her cerebellar examination showed slowing of rapid alternating movements in the left hand. Magnetic resonance imaging of the lumbosacral spine indicated subtle T2 signal change of the intramedullary conus and enhancement of the cauda equina nerve roots. Cerebrospinal fluid analysis showed an increased protein concentration. Two white blood cells/µL were found in the cerebrospinal fluid. The serum antinuclear antibody was strongly positive, and the anti–double-stranded DNA antibody level was greater than 1,000 IU/mL. The serum complement levels were low. Lupus anticoagulant, beta-2 glycoprotein antibodies, and antiphospholipid antibodies were increased, at greater than twice the upper limits of normal. Electromyography indicated multiple sacral radiculopathies. The patient was diagnosed with autoimmune myeloradiculitis as a neuropsychiatric manifestation of systemic lupus erythematosus (neuropsychiatric systemic lupus erythematosus). The patient received methylprednisolone followed by prednisone, with a gradual taper. Her hospital course was complicated by the development of deep venous thromboses in the bilateral lower extremities. She was started on heparin and transitioned to warfarin therapy. She started mycophenolate mofetil. Hydroxychloroquine was continued. At a 24-month follow-up visit, the patient remained in neurologic remission. Neuropsychiatric systemic lupus erythematosus events consist of a heterogeneous array of neurologic and psychiatric disorders including intractable headaches, cognitive dysfunction, psychosis, seizure disorders, transverse myelitis, aseptic meningitis, cranial neuropathies, and acute inflammatory demyelinating polyneuropathy.

COVID-19 neurological manifestations: correlation of cerebral MRI imaging and lung imaging—Observational study

Background During the current pandemic, there is an increased incidence of neurologic/neuropsychiatric manifestations in patients with the novel coronavirus (COVID-19). Neurologic manifestations may be coincident or result of disease and its therapy. In the emergency department, orientation of the clinician with this issue is crucial for accurate decision making to limit the spread of infection during neurologic treatment. This study aimed to be familiar with MRI findings in patients with Neuro-COVID. Seventy patients presented with neurologic/neuropsychiatric manifestation either post COVID or during hospitalization underwent cerebral MRI from April 2020 to June 2021 (39 men and 31women; mean age 43.27, age range from 16 to 69 years). Results Headache (80%), is the most prevalent neurological manifestations followed by smell and taste impairment (62.9%) and stroke symptoms (45.7%). Low mood and anxiety (17.1%), prolonged fatigue (14.3%) and depression (7.1%) are the most common psychiatric symptoms. Infarctions, hematoma and demyelinating disease are the most prevalent findings. There is a week positive correlation between MRI findings and CT chest finding but without statistical significance (P-value 0.2). Conclusions Cerebrovascular disease and demyelinating lesions are common manifestations of COVID 19. Familiarity of neurologists and radiologist in the emergency department and in-patient with this issue is crucial to avoid misdiagnosis and the spread of infection.

Cushing’s syndrome manifesting as chronic insomnia caused by adrenal cortical adenoma with incidental pituitary microadenoma: a case report

Background: Cushing’s syndrome is condition caused by excessive glucocorticoid with insomnia as one of its neuropsychiatric manifestation. Cushing’s syndrome may be caused by excessive adrenocorticotropin hormone (ACTH-dependent), for example from ACTH producing pituitary tumors, or by overproduction of cortisol by adrenocortical tumors. In this report, we presented a case with Cushing’s syndrome manifesting as chronic insomnia with adrenal cortical adenoma and pituitary microadenoma.Case presentation: A 30-year-old woman was consulted from the Neurologic Department to the Internal Medicine Department with the chief complaint of insomnia and worsening headache since 6 months prior to the admission. She had undergone head MRI and abdominal CT scan previously and was found to have both pituitary microadenoma and left adrenal mass. From the physical examination she had clinical signs of Cushing’s syndrome like Cushingoid face and purplish striae on her stomach. Midnight cortisol serum examination was done initially and showed high level of cortisol. High dose dexamethasone suppression test or DST (8 mg overnight) was later performed to help determine the main cause of Cushing’s syndrome. The result failed to reach 50% suppression of cortisol serum, suggestive that the Cushing’s syndrome was not ACTH-dependent from the pituitary but potentially from overproduction of cortisol by the left adrenal mass. Therefore, left adrenalectomy was performed and the histopathological study supported the diagnosis of adrenal cortical adenoma.Conclusion: Chronic insomnia is a very important symptoms of Cushing’s syndrome that should not be neglected. The patient had both microadenoma pituitary and left adrenal mass thus high dose DST test (8 mg overnight) needed to be performed to differentiate the source of Cushing’s syndrome. The result showed only little suppression therefore the pituitary microadenoma was not the source of Cushing’s syndrome and more suggestive from the adrenal etiology.

Isolated extrapontine myelinolysis with neuropsychiatric manifestation: A case report from Nepal

A referred case of a 34-year-old woman was brought to the emergency room with features of mutism and whole-body rigidity following rapid correction of hyponatremia. On presentation, she was ill looking with tremors on her both hands and legs. She was unresponsive to command and developed involuntary outbursts of laughter and crying. Based on clinical features and neuroimaging, diagnosis of isolated extrapontine myelinolysis was made. She was treated with quetiapine, vitamin B1 and B12 supplements, trihexyphenidyl, levodopa-carbidopa and physiotherapy of limbs. Due to lack of clinical trials for adequate diagnosis and management of extrapontine myelinolysis, this case report highlights the importance of extrapontine myelinolysis with neuropsychiatric manifestation in research world.

MANIFESTASI NEUROPSIKIATRIK PADA LUPUS ERITEMATOSUS SISTEMIK DI RSUD DR. SOETOMO SURABAYA

NEUROPSYCHIATRIC MANIFESTATION AMONG SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS IN GENERAL HOSPITAL SURABAYAABSTRACTIntroduction: Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease with unknown causes and extensive clinical manifestations and diverse disease pathways. The clinical manifestations of SLE are very diverse, including the involvement of the nervous system and psychiatric syndrome (neuropsychiatric).Aims: To describe clinical neuropsychiatric manifestations of patients with SLE in Soetomo General Hospital Surabaya.Methods: This was a cross-sectional study based on medical record data on all SLE patients treated at Dr. Soetomo Hospital Surabaya, from January-December 2017. Neuropsychiatric manifestations assessment were based on the nomenclature of the American College of Rheumatology (ACR) in 1999.Results: There were 49 patients, mostly women (98%) with mean age 30,8±10,2 years old. Neurological manifestation was the 3rd most common diagnosis (43%) after hematologic disorder (73.5%) arthritis (53.1%). The manifestations of neuropsychiatric manifestation were mainly seizures (40.8%), headache (34.7%), cerebrovascular disease (26.5%), acute confusional state (20.4%), cognitive dysfunction (6.1%), and polyneuropathy (8.2%).Discussion: The most common neuropsychiatric clinical features in SLE patients are seizures, headache, cerebrovascular disease, and acute confusional state.Keywords: Lupus neuropsychiatry, prevalence, systemic lupus erythematosusABSTRAKPendahuluan: Lupus eritematosus sistemik (LES) merupakan penyakit inflamasi autoimun kronik yang belum diketahui penyebabnya dengan perjalanan penyakit yang luas. Manifestasi klinis LES sangat beragam, antara lain keterlibatan sistem saraf dan sindrom psikiatri (neuropsikiatri).Tujuan: Untuk mengetahui manifestasi neuropsikiatrik pasien dengan LES di RSUD Dr. Soetomo, Surabaya.Metodologi: Penelitian potong lintang berdasarkan data rekam medik terhadap semua pasien LES yang dirawat di RSUD Dr. Soetomo, Surabaya, pada bulan Januari hingga Desember 2017. Manifestasi neuropsikiatrik dinilain berdasarkan nomenklatur American College of Rheumatology (ACR) tahun 1999.Hasil: Didapatkan 49 subjek yang hampir semuanya (98%) adalah perempuan dengan rerata usia 30,8±10,2 tahun. Gangguan neurologis merupakan ketiga tersering (43%) setelah gangguan hematologi (73,5%) dan artritis (53,1%). Manifestasi neuropsikiatri terutama kejang (40,8%), nyeri kepala (34,7%), penyakit serebrovaskular (26,5%), keadaan konfusi akut (20,4%), dan polineuropati (8,2%).Diskusi: Manifestasi klinis neuropsikiatri yang paling banyak dialami oleh pasien LES adalah kejang, nyeri kepala, penyakit serebrovaskular, dan keadaan konfusi akut.Kata kunci: Lupus eritematosus sistemik, manifestasi klinis, neuropsikiatri

Child and adolescent neuropsychiatry

Child neuropsychiatry encompasses childhood and adolescent psychiatric syndromes of neurobiological origins. It is an evolving discipline without consensus on its exact boundary. Given the inconsistencies, this chapter provides a historical perspective through which different conceptualizations of child neuropsychiatry can be understood and reconciled within the coherent whole. Four specific contrasting conditions are selected in this chapter to illustrate some key principles: attention-deficit/hyperactivity disorder (ADHD) as ‘a diffuse brain disorder’; childhood cranial tumours as ‘a localized brain disorder’; fetal alcohol spectrum disorders (FASD) as ‘a disorder of a specific cause’ represented by toxin exposure; and epilepsy as ‘a disorder of complex aetiology’. ADHD and cranial tumours represent the extremes of the polar divide between ‘a childhood neuropsychiatric disorder’ and ‘the neuropsychiatric manifestation of a childhood neurological disorder’. In contrast, FASD and epilepsy illustrate how specific and complex aetiology can present with a wide spectrum of psychiatric disorders. Atypical presentations of psychiatric symptoms, idiosyncratic treatment response, and ‘diagnostic overshadowing’ are also considered. The chapter emphasizes that child neuropsychiatric conditions are not fixed entities, despite conforming to diagnostic criteria stipulated by authoritative taxonomic systems. Rather, they are the results of the dynamic interplay between environmental factors, developmental maturity, mitigating factors, aberrant neural networks, and innate disease liability.