eNeurologicalScieNeurologicalSci Vol 18

March 2020

Original Articles

Rhizomelic chondrodysplasia punctata: Role of EEG as a biomarker of impending epilepsy

Debopam Samanta

  • Article 100218
  • https://doi.org/10.1016/j.ensci.2019.100218
  • Download PDF
  • Abstract

    Progressive deterioration of neuroimaging and electroencephalography (EEG) had been described in rhizomelic chondrodysplasia punctata (RCDP); however, serial EEG data showing sequential EEG changes(before and after seizure onset) is lacking. We report a child with a diagnosis of type 1 RCDP, who had a progressive decline in EEG and radiologic findings over a 5 year period. Her first EEG was normal at the age of 8 months. Follow-up EEG at the age of 2 years showed a mild background slowing as well as frequent 1–2 Hz central-parietal spike wave with midline involvement. Just before 3 years of age, she started to seizures, when the EEG showed further worsening with frequent multifocal spikes and bursts of generalized high amplitude spike and spike-wave discharges. The transition of EEG from normal background to the appearance of focal epileptiform abnormality before the seizure onset followed by further deterioration at the seizure onset had not been reported as per our knowledge. This study emphasizes that serial EEGs may provide valuable information about impending seizure activity. Further studies are needed to calculate the lag time between the detection of epileptiform activities and the onset of clinical seizure activities. In addition, research studies are warranted to determine if early (before or at the onset of epileptogenesis rather than after seizure onset) use of replacement therapy or antiepileptic therapy (antiepileptic drugs or diet) can modify epilepsy severity and neurologic prognosis in this devastating disease.

Serum B-type natriuretic peptide level and timing of its measurement as a predictor of acute ischemic stroke outcome

Kousuke Fukuhara, Toshiyasu Ogata, Sho Takeshita, Yoshio Tsuboi

  • Article 100217
  • https://doi.org/10.1016/j.ensci.2019.100217
  • Download PDF
  • Abstract

    An increased B-type natriuretic peptide (BNP) level is associated with cardioembolic stroke because of atrial fibrillation. However, data associating the measurement time of BNP and clinical influence of BNP are limited. Herein, we examined the utility of BNP level for prediction of stroke severity when accounting for measurement time. We retrospectively registered 327 patients admitted within 7 days from onset of ischemic stroke. We collected information on patients' background, stroke risk factors, subtype and severity, and outcome at discharge. Measurement of BNP was performed by chemiluminescent enzyme immunoassay. Patients were divided into 3 groups according to the time of BNP measurement from disease onset. Multivariate analyses were performed to evaluate the association of BNP value with outcome after patients were grouped according to BNP measurement time. Of the 327 patients, the numbers of patients whose BNP was measured within 24 h of symptom onset, between 24 and 48 h of symptom onset, and after 48 h of symptom onset were 102, 92, and 133, respectively. Favourable outcome at discharge was negatively correlated with BNP value in patients with a BNP level measured within 24 h of stroke onset. BNP value may be useful for prediction of stroke outcome if measured within 24 h after stroke onset.

Long-term outcome of resuming anticoagulation after anticoagulation-associated intracerebral hemorrhage

Alireza Sadighi, Lisa Wasko, Heather DiCristina, Thomas Wagner, ... Ramin Zand

  • Article 100222
  • https://doi.org/10.1016/j.ensci.2020.100222
  • Download PDF
  • Abstract

    The risk and benefit of restarting oral anticoagulation (OAC) therapy among patients with atrial fibrillation or flutter (AF) and an episode of anticoagulation-associated intracerebral hemorrhage (ICH) remain unclear. Whether or not to resume OAC after an OAC-associated ICH will remain an unanswered clinical question until we have sufficient data through randomized clinical trials. Here, we analyzed the long-term outcome of patients with AF who did or did not resume OAC after an OAC-associated ICH.

Premorbid instrumental activities of daily living predicts discharge home following stroke

Takeshi Satow, Taro Komuro, Masafumi Ogawa

  • Article 100226
  • https://doi.org/10.1016/j.ensci.2020.100226
  • Download PDF
  • Abstract

    Stroke survivors who remain dependent require multiple healthcare resources, including rehabilitation and nursing care. The effect of premorbid instrumental ADL (IADL) on the discharge destination, which has not been studied previously in detail, is analyzed.


Case Reports

Treatment-related fluctuations in subacute inflammatory demyelinating polyneuropathy

Young Gi Min, Yoon-Ho Hong

  • Article 100224
  • https://doi.org/10.1016/j.ensci.2020.100224
  • Download PDF
  • Abstract

    Treatment-related fluctuation (TRF), only defined in Guillain-Barre syndrome (GBS), refer to the deterioration of symptoms following treatment-induced improvement, and implies disease activity lasting beyond the effect of immunotherapy. Here, we first propose the concept of TRF in subacute inflammatory demyelinating polyneuropathy (SIDP) with description of a corresponding case. A 27-year-old female presented with acute flaccid paralysis, and experienced two sequential episodes of TRF, the latter occurring around 8 weeks from disease onset. She eventually recovered through intravenous immunoglobulin treatment, and has not experienced any further deterioration over the next four years. The concept of SIDP-TRF would resolve the gap between GBS-TRF and acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and help to decide the optimal treatment strategy in a spectrum of idiopathic immune-mediated polyneuropathies.

Can osmotic demyelination syndrome be a complication of liver failure?

Kaylynn Purdy, Dustin Anderson, Richard Camicioli, Rachel G. Khadaroo

  • Article 100223
  • https://doi.org/10.1016/j.ensci.2020.100223
  • Download PDF
  • Abstract

    This case demonstrates that osmotic demyelination syndrome (ODS) can occur in absence of hyponatremia in patients with fulminant liver failure and markedly high bilirubin levels. Extremely high bilirubin levels, such as >900 μmol/L in the case presented here, may lead to blood brain barrier dysfunction by disrupting blood vessel endothelial cell function as well as increase the release of inflammatory cytokines. As demonstrated in the case here, even small fluctuations in electrolytes may make the brain increasingly more vulnerable to ODS. Clinicians should keep ODS high on their differential even in eunatremic patients with liver failure who have decreased levels of consciousness or coma.

A case of seronegative longitudinally extensive transverse myelitis with possible neuro sweet disease

Megumi Toko, Tomohisa Nezu, Hiroki Ueno, Teppei Kamimura, ... Hirofumi Maruyama

Nasopharyngeal carcinoma spinal metastasis without percussion tenderness

Kai-Chieh Chang, Hsueh-Wen Hsueh

Intravenous metoclopramide induced generalized multifocal myoclonus

Luís Ribeiro, Ana Monteiro


Letters to the Editor

Is "delayed" hyperbaric therapy effective for "delayed" encephalopathy after carbon monoxide intoxication?

Irene Pappalardo, Lorenzo Celli, Luciano Arena, Lucia Testa, ... Eugenia Rota

Reversible splenial lesion in a patient with new-onset refractory status epilepticus (NORSE)

Akane Unno Mizutani, Akihiro Shindo, Shigeo Arikawa, Takuya Shimada, ... Hidekazu Tomimoto

  • Article 100220
  • https://doi.org/10.1016/j.ensci.2019.100220
  • Download PDF
  • Abstract

    New-onset refractory status epilepticus (NORSE) is a rare neurological emergency condition with poor prognosis. A 30-year-old male suddenly had tonic-clonic convulsions seven days after a preceding fever and diarrhea. MRI showed a reversible splenial lesion, and he developed refractory multifocal and generalized seizures in spite of anticonvulsant medication. He was diagnosed with NORSE and received a combination treatment with immunotherapy and targeted temperature management (TTM), which effectively decreased his seizures. This case suggests that even for patients with reversible splenial lesions, NORSE should be considered, and that treatment with immunotherapy and TTM may be effective.

Pediatric critical illness associated cerebral microhemorrhages

Anna Misyail Abdul Rashid, Norafida Bahari, Mohamad Syafeeq Faeez Md Noh

Abnormal evoked potentials in autoimmune glial fibrillary acidic protein astrocytopathy

Ryo Tokimura, Nozomu Matsuda, Shunsuke Kobayashi, Akio Kimura, Kazuaki Kanai

  • Article 100229
  • https://doi.org/10.1016/j.ensci.2020.100229
  • Download PDF
  • Abstract

    Autoimmune GFAP astrocytopathy is a new clinical entity and only a limited number of cases have been reported. Here we report the results of multimodal central conduction studies performed in a case of this disorder.

    A 72-year-old woman developed gradual cognitive decline and gait disturbance. A neurological examination revealed moderate amnesia, papilloedema, and pyramidal tract impairment of the bilateral lower limbs. The diagnosis of autoimmune GFAP astrocytopathy was made based on the typical MRI findings of periventricular radial linear gadolinium enhancement in the brain and longitudinally extensive lesions in the spinal cord, and anti-GFAP antibody detected in the cerebrospinal fluid. Somatosensory evoked potentials and transcranial magnetic stimulation studies revealed prolongation of conduction times. Visual evoked potentials showed an unusual W-shaped pattern. To our knowledge, this is the first neurophysiological demonstration of prolonged central conduction times in the autoimmune GFAP astrocytopathy. Further investigations are needed to establish the clinical value the neurophysiological examinations in this disorder.

Kampō medicine and Muro disease (Amyotrophic Lateral Sclerosis and Parkinsonism-Dementia Complex)

Peter S. Spencer, Valerie S. Palmer, Tameko Kihira, Sohei Yoshida, ... Yoshiro Yase

  • Article 100230
  • https://doi.org/10.1016/j.ensci.2020.100230
  • Download PDF
  • Abstract

    Western Pacific Amyotrophic Lateral Sclerosis and Parkinsonism-dementia Complex (ALS/PDC) is a disappearing neurodegenerative disease in three former high-incidence foci of Guam-USA, Papua-Indonesia and Kii Peninsula, Honshu Island, Japan. The latter includes two distinct ALS/PDC-affected regions (Hohara and Kozagawa), where the disorder is known as Muro disease. In Hohara, oral exposure to plant (cycad) neurotoxins used in traditional medical practice has been linked previously to Muro disease. We report new observations that link Kampō medicine to Muro disease in the southern Kozagawa focus. Oral exposure to cycad seed toxins is associated with all three foci of Western Pacific ALS/PDC.

Cerebellar stroke-like lesions in Leigh syndrome due to the variant m.8993T>C in MT-ATP6

Josef Finsterer

Leigh Syndrome with atypical cerebellum imaging features

Marcos Gil Alberto da Veiga, Clara Marecos, José Pedro Vieira, Carla Conceição