eNeurologicalScieNeurologicalSci Vol 35

June 2024

Review Article

Transcranial direct current stimulation for chronic foot pain: A comprehensive review

Roberto Tedeschi

  • Article 100498
  • https://doi.org/10.1016/j.ensci.2024.100498
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  • Abstract

    Chronic foot pain, including conditions such as plantar fasciitis, presents a significant challenge to patients and healthcare providers. Traditional treatments often offer limited relief, prompting exploration of alternative therapies. Transcranial direct current stimulation (tDCS) has emerged as a noninvasive brain stimulation technique with potential for alleviating chronic pain syndromes.

    A review was conducted following the JBI methodology and adhering to PRISMA guidelines. Searches were performed in databases including MEDLINE, Cochrane Central, Scopus, and PEDro, supplemented by grey literature sources and expert consultations. Studies were included if they investigated tDCS as an intervention for chronic foot pain, assessed its efficacy, safety, or mechanisms of action, and were published in English.


Original Articles

Effect of moyamoya disease on the basilar artery and adjacent arteries on CTA

Han Su, Jinlu Yu

  • Article 100501
  • https://doi.org/10.1016/j.ensci.2024.100501
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  • Abstract

    Computed tomographic angiography (CTA) is rarely used to explore the effect of moyamoya disease (MMD) on the basilar artery (BA) and its adjacent arteries.

    Participants were divided into a control group and an MMD group. The relevant parameters were measured. Statistical analyses included the t-test, chi-squared test, and linear regression analysis.

Corrected speciation and gyromitrin content of false morels linked to ALS patients with mostly slow-acetylator phenotypes

Emmeline Lagrange, Marie-Anne Loriot, Nirmal K. Chaudhary, Pam Schultz, ... Peter S. Spencer

  • Article 100502
  • https://doi.org/10.1016/j.ensci.2024.100502
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  • Abstract

    A case-control study of sporadic amyotrophic lateral sclerosis (ALS) in a mountainous village in the French Alps discovered an association of cases with a history of eating wild fungi (false morels) collected locally and initially identified and erroneously reported as Gyromitra gigas. Specialist re-examination of dried specimens of the ALS-associated fungi demonstrated they were members of the G. esculenta group, namely G. venenata and G. esculenta, species that have been reported to contain substantially higher concentrations of gyromitrin than present in G. gigas. Gyromitrin is metabolized to monomethylhydrazine, which is responsible not only for the acute oral toxic and neurotoxic properties of false morels but also has genotoxic potential with proposed mechanistic relevance to the etiology of neurodegenerative disease. Most ALS patients had a slow- or intermediate-acetylator phenotype predicted by N-acetyltransferase-2 (NAT2) genotyping, which would increase the risk for neurotoxic and genotoxic effects of gyromitrin metabolites.

The effects of lactulose on constipation in patients with Parkinson's disease: An exploratory pilot study

Shin-ichiro Kubo, Mako Ito, Kyoko Matsuba, Tomohiro Shimono

  • Article 100503
  • https://doi.org/10.1016/j.ensci.2024.100503
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  • Abstract

    Constipation is one of the most common non-motor symptoms of Parkinson's disease (PD) and is associated with reduced quality of life in patients with PD. The aim of this study was to evaluate the effect of lactulose on defecation status in patients with PD.

    In this open-label, single-center, exploratory pilot study, twenty-nine patients with PD received lactulose for three weeks for the treatment of constipation. The primary endpoint was the number of spontaneous bowel movements (SBMs). The secondary endpoints were stool consistency (Bristol Stool Form Scale [BSFS]) and the number of rescue laxatives used.


Case Reports

Phenotypic variability in a large kindred with spastic paraplegia associated with a novel REEP1 variant

Helgi Thor Hjartarson, Humberto Skott, Tobias Granberg, Martin Paucar

  • Article 100497
  • https://doi.org/10.1016/j.ensci.2024.100497
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  • Abstract

    The aim of this study is to provide a comprehensive characterization of a large Estonian family spanning five generations with seventeen individuals affected by spastic paraplegia associated with a novel variant in the receptor expression-enhancing protein-1 (REEP1) gene.

    Comprehensive clinical evaluation, neuroimaging, and neurophysiological studies were performed on six patients who provided oral and written consent. Whole-exome sequencing was performed on the index case. Targeted carrier testing was done in all other available affected and at-risk relatives.

CASE REPORT: Fulminant acute hemorrhagic Leukoencephalitis (AHLE): A rare and ruinous outcome with cerebral herniation (COVID-19)

Abeer Sabry Safan, Zeba Noorain, Mohamed A. Atta, Razna Thekkoth, ... Mohammed Abdelatey

  • Article 100499
  • https://doi.org/10.1016/j.ensci.2024.100499
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  • Abstract

    Acute hemorrhagic leukoencephalitis (AHLE) is a very rare demyelinating disease with rapid fulminant inflammation of the white matter. Although the exact etiology is unknown, AHLE usually manifests post a viral or bacterial infection and less often seen post vaccination for measles or rabies. AHLE has a very poor prognosis and a high mortality rate. Owing to the rarity of this entity there is not clear consensus on the proper line of management. In this report, we present a case of AHLE as a para-infectious sequel to COVID-19 in a young patient.

    We report a 30-year-old turkish patient with an initial presentation of upper respiratory tract infection due to COVID-19. Initially, she was admitted to the hospital with generalized tonic-clonic seizure (GTCS) and deterioration in her level of consciousness lapsing into a coma. An initial CT scan showed diffuse brain edema and an MRI head confirmed the suspicion of Acute hemorrhagic leukoencephalitis (AHLE). Despite prompt and diligent osmotic therapy and pulsed intravenous (IV) methylprednisolone, her condition rapidly depreciated and progressed into cerebral edema with gravid sequela of brainstem herniation.

Distal oesophageal spasm in a patient with multiple system atrophy: A case report

Yoya Ono, Kenjiro Kunieda, Jun Takada, Takayoshi Shimohata

  • Article 100500
  • https://doi.org/10.1016/j.ensci.2024.100500
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  • Abstract

    A 74-year-old man developed orthostatic syncope, a feeling of food stuck in his chest, and postprandial vomiting 3 years before presentation. Examination revealed severe orthostatic hypotension and cerebellar ataxia, and he was diagnosed with multiple system atrophy (MSA) with predominant cerebellar ataxia. Videofluoroscopic examination of swallowing showed lower oesophageal stricture and barium stagnation within the oesophagus. Oesophagogastroduodenoscopy revealed hypercontraction of the lower oesophagus, and high-resolution oesophageal manometry showed premature contractions of the lower oesophagus and decreased oesophageal peristalsis. The median integrated relaxation pressure in the lower oesophageal sphincter was normal, and achalasia was therefore excluded. Based on the Chicago classification version 4.0, his oesophageal dysmotility was classified as distal oesophageal spasm (DES). The stuck feeling in his chest and vomiting improved following endoscopic balloon dilation. This case suggests that DES can cause oesophageal food stagnation and postprandial vomiting in patients with MSA.

Bing Neel syndrome presenting as isolated cranial nerve palsies – a case report

Dipti Baskar, Davuluri Durga Srinivas Anudeep, Seena Vengalil, Preetham Patavaradhan, ... Atchayaram Nalini

  • Article 100505
  • https://doi.org/10.1016/j.ensci.2024.100505
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  • Abstract

    Waldenstroms macroglobulinemia (WM) is a low-grade B cell neoplasm. Bing Neel syndrome is a rare manifestation of WM characterized by infiltrative involvement of the central nervous system.

    64-year-old man, presented with 4 years history of slowly progressive diplopia and ptosis of eyes. Examination showed left oculomotor (internal and external ophthalmoplegia), with trochlear, abducens, and right partial oculomotor and abducens nerve involvement. Evaluation showed anemia of hemoglobin 10.7 g/dL, raised erythrocyte sedimentation rate of 120 mm/h and plasma albumin:globulin reversal. Serum protein electrophoresis showed a paraprotein peak in the early gamma region with elevated IgM level (3810 mg/dL) and elevated free kappa light chain level (70.1 mg/L). Bone marrow aspiration from posterior iliac crest revealed mature small lymphocytes with positive immunohistochemical markers of CD5, CD10 negativity and MYD88 mutation positivity suggestive of WM. Patient was treated with bendamustine and rituximab regimen, with no neurological improvement at the end of one year.

    This case expands spectrum of paraproteinemic neuropathy to include cranial nerve palsy. Thus, plasma cell dyscrasias have to be considered in patients with isolated ophthalmoparesis especially in elderly patients, even with other comorbidities such as diabetes mellitus.

Spinal melanoma with optic neuropathy –rare manifestation of Neurocutaneous melanosis and PET-MRI findings

Dipti Baskar, Seena Vengalil, Priyanka Chakkera, Sai Bhargava Sanka, ... Atchayaram Nalini

  • Article 100504
  • https://doi.org/10.1016/j.ensci.2024.100504
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  • Abstract

    Neurocutaneous melanocytosis (NCM) is a rare, sporadic neuroectodermal dysplasia characterized by the presence of large or multiple congenital cutaneous nevi and melanocytic deposits in the central nervous system. Hitherto, unreported we describe a case of NCM with optic neuropathy and spinal cord melanoma from India.

    A 20 year-old-lady had headache and vomiting for 3 months followed by consecutive profound painless visual impairment. Visual acuity was counting of fingers at 1 m distance in both eyes with normal fundus. There were no symptoms of spinal cord involvement. Clinical examination showed multiple small to large melanocytic nevi over the face and body. Muscle power was normal. Tendon reflexes were exaggerated. Visual evoked potential showed bilateral prolonged P100 latency (Right eye - 144 msec; Left eye - 151 msec). Brain MRI revealed leptomeningeal enhancement of brainstem, cerebellum, oculomotor and facial-abducent nerve complex without optic nerve involvement. MRI spine showed extensive dorsal thoracic cord epidural lesion extending along the entire thoracic cord segment with dorsal cord compression. Positron Emission Tomography (PET) imaging showed Fludeoxyglucose F18 (FDG) avidity along D1-D12 levels of spinal cord. Biopsy from the cord lesion was suggestive of meningeal melanoma. Here we document a rare case of late onset NCM with intracranial meningeal infiltration and asymptomatic large epidural lesion of spinal cord, expanding its phenotypic spectrum. Optic neuropathy in NCM has not been reported earlier. Periodic screening of brain and spine is recommended for early prognostication and lesion identification in NCM

A novel SPAST gene splicing variant (c.1617-2A>C) in a heterozygous carrier with hereditary spastic paraplegia

Elvira Sbragia, Andrea Assini, Silvia Calzavara, Paola Carrera, ... Emilio Di Maria

  • Article 100506
  • https://doi.org/10.1016/j.ensci.2024.100506
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  • Abstract

    Hereditary spastic paraplegia (HSP) is a group of genetically heterogenous neurodegenerative disorders characterized by progressive spasticity and weakness of lower limbs. We report a novel splicing variant (c.1617-2A>C) of the SPAST gene in a heterozygous carrier from an Italian family with autosomal dominant HSP. The case study describes a pure form of spastic paraparesis with the cardinal clinical features of SPG4. The novel variant affects a canonical splice site and is likely to disrupt RNA splicing. We conclude that the c.1617-2A>C substitution is a null variant, which could be classified as pathogenic; its penetrance should be further investigated

A decade with anomic primary progressive aphasia

Shoko Ota, Kazuo Kakinuma, Wataru Narita, Yoshiyuki Nishio, ... Kyoko Suzuki

  • Article 100508
  • https://doi.org/10.1016/j.ensci.2024.100508
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  • Abstract

    Some patients with primary progressive aphasia (PPA) demonstrate only anomia. The lack of longitudinal observations of anomic PPA precluded us from determining whether progressive anomic aphasia was simply an early stage of semantic or logopenic variants, or a relatively independent variant. Herein, we report the 10-year clinical course of a patient with PPA who presented with pure anomic aphasia for 9 years. He is a right-handed man with anomia, who noticed word-finding difficulty at age 73. He was admitted to the hospital at age 77. On admission, the patient showed pure anomic aphasia with preserved other language function. Episodic memory and visuospatial function were preserved. Magnetic resonance imaging (MRI) revealed left temporal lobe atrophy. At 82 years of age, the patient presented with pure anomic aphasia. At 83 years old, he showed mild impairment in word comprehension and semantic memory, in addition to anomia. MRI demonstrated further atrophy in the bilateral anterior temporal lobes, predominantly on the left side. This case suggests the possibility of slowly progressive, late-onset anomic PPA, which could be differentiated from the early stage of semantic or logopenic variants.


Letters to the Editor

Unforeseen effects: Hiccups unveiled by high-dose steroids in demyelination

Sai Niharika Tammineedi, Ramit Singla, Marilhia Cornejo Leon, Muskan Kohli, ... Aakanksha Pitiliya

  • Article 100509
  • https://doi.org/10.1016/j.ensci.2024.100509
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  • Abstract

    Intractable hiccups, persisting beyond 48 h, pose a clinical challenge, particularly in demyelinating diseases like Neuromyelitis Optica (NMO) and Multiple Sclerosis (MS). Understanding the complex neural pathways of the hiccup reflex and the impact of high-dose steroid therapy is crucial for managing this rare but distressing symptom. The hiccup reflex involves afferents from the vagus, phrenic, and sympathetic nerves, with the reflex center in the anterior horns at the C3 to 5 level and the medulla oblongata. The potential interplay between demyelination and corticosteroid therapy in triggering persistent hiccups requires exploration.

    This case report details a 21-year-old male with undiagnosed demyelinating disorder, presenting persistent hiccups following high-dose steroid therapy for an acute disease flare. The patient's history included vertigo and progressive neurological symptoms, leading to an MS diagnosis with significant brain and spinal lesions. Persistent hiccups, initiated by steroid administration, were recurrent but responsive to metoclopramide after other measures failed.

    The discussion centers on investigating the cause of hiccups in a patient with demyelination following steroid administration. Steroids' impact on neurological systems, including neurotransmitter function, and the potential disruption of neurological pathways due to demyelination may contribute to hiccups. Successful hiccup resolution with metoclopramide suggests a potential pharmacological approach for corticosteroid-induced hiccups in demyelinating diseases. This case emphasizes the need for further research into the intricate relationship between demyelination, steroid therapy, and hiccups to enhance management strategies for this uncommon yet impactful symptom.

A case of chronic progressive autoimmune GFAP astrocytopathy with extensive meningoencephalomyelitis and contrast enhancement on MRI

Hironori Oka, Takumi Nakamura, Takashi Sugawara, Kunihiko Ishizawa, ... Mikio Shoji