Publications by authors named "Hindi Alhindi"

6 Publications

  • Page 1 of 1

Frontal disconnection surgery for drug-resistant epilepsy: Outcome in a series of 16 patients.

Epilepsia Open 2020 Sep 14;5(3):475-486. Epub 2020 Aug 14.

King Faisal Specialist Hospital & Research Center Riyadh Saudi Arabia.

Objective: To evaluate the effectiveness of frontal disconnection surgery in seizure control and related consequences in a consecutive patient series.

Methods: We conducted a retrospective analysis of patients who underwent frontal disconnection surgery for drug-resistant epilepsy (DRE). Baseline epilepsy characteristics, detailed presurgical evaluation including epileptogenic zone (EZ) localization, magnetic resonance imaging (MRI) detection of epileptogenic lesion, and pathological findings were reviewed. Patients were followed postoperatively for seizure outcome at 1 year.

Results: A total of 16 patients were identified (six children and 10 adults). Most patients had a childhood onset of DRE with a median duration of epilepsy of 6.5 years (interquartile range 3.5-17.5 years) before surgery. In 10 (62.5%) patients, the EZ was localized to the frontal lobe, while in six patients, the EZ involved also adjacent lobes or consisted of multiple foci. In 10 (62.5%) patients, an epileptogenic lesion was detected on presurgical MRI, four of which (40%) had all MRI abnormalities confined to the frontal lobe. Two-thirds of the patients (11/16; 68.8%) underwent isolated frontal disconnection procedure, while remaining patients had frontal disconnection combined with resection of an adjacent lobe. Of the 12 patients in whom biopsy was taken from the disconnected frontal lobe, six (50%) had pathology-proven focal cortical dysplasia. We observed surgical-related complications in three (18.8%) cases, neurological deficits in other three (18.8%) patients, and worsening cognitive abilities in one (6.3%) patient. Overall, eight (50%) patients became completely seizure-free (ILAE 1) at one-year follow-up.

Significance: Frontal disconnection surgery for DRE can result in seizure freedom in certain patients, especially when the EZ is strictly limited to the ipsilateral frontal region, and the MRI shows an epileptogenic lesion that is purely frontal in location. Frontal lobe disconnection procedure is safe and has a limited complication rate. However, further studies with larger patient population will yield more significance.
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September 2020

New onset refractory status epilepticus due to primary angiitis of the central nervous system.

Epilepsy Behav Case Rep 2017 19;8:100-104. Epub 2017 Aug 19.

Department of Neurosciences, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia.

Primary Angiitis of the central nervous system is a rare and poorly understood variant of vasculitis. We narrate a case of a 46-year-old male who presented with new onset refractory status epilepticus mimicking autoimmune encephalitis. In this case we are reporting clues that could be useful for diagnosis and extensive literature review on the topic.
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August 2017

A first-line diagnostic assay for limb-girdle muscular dystrophy and other myopathies.

Hum Genomics 2016 Sep 27;10(1):32. Epub 2016 Sep 27.

Saudi Human Genome Program, King Abdulaziz City for Science and Technology, Riyadh, Saudi Arabia.

Background: Fifty random genetically unstudied families (limb-girdle muscular dystrophy (LGMD)/myopathy) were screened with a gene panel incorporating 759 OMIM genes associated with neurological disorders. Average coverage of the CDS and 10 bp flanking regions of genes was 99 %. All families were referred to the Neurosciences Clinic of King Faisal Specialist Hospital and Research Centre, Saudi Arabia. Patients presented with muscle weakness affecting the pelvic and shoulder girdle. Muscle biopsy in all cases showed dystrophic or myopathic changes. Our main objective was to evaluate a neurological gene panel as a first-line diagnostic test for LGMD/myopathies.

Results: Our panel identified the mutation in 76 % of families (38/50; 11 novel). Thirty-four families had mutations in LGMD-related genes with four others having variants not typically associated with LGMD. The majority of cases had recessive inheritance with homoallelic pathogenic variants (97.4 %, 37/38), as expected considering the high rate of consanguinity in the study population. In one case, we detected a heterozygous mutation in DNAJB responsible for LGMD-1E. Our cohort included seven different subtypes of LGMD2. Mutations of DYSF were the most commonly identified cause of disease followed by that in CAPN3 and FKRP. Non-LGMD myopathies were due to mutations in genes associated with congenital disorder of glycosylation (ALG2), rigid spine muscular dystrophy 1 (SEPN1), inclusion body myopathy2/Nonaka myopathy (GNE), and neuropathy (WNK1). Whole exome sequencing (WES) of patients who remained undiagnosed with the neurological panel did not improve our diagnostic yield.

Conclusions: Our neurological panel achieved a high clinical sensitivity (76 %) and is an effective first-line laboratory test in patients with LGMD and other myopathies. This sensitive, cost-effective, and rapid assay significantly assists clinical practice especially in these phenotypically and genetically heterogeneous disorders. Moreover, the application of the American College of Medical Genetics (ACMG) and Association for Molecular Pathology (AMP) guidelines applied in the classification of variant pathogenecity provides a clear interpretation for physicians on the relevance of such findings.
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September 2016

Clinical and genetic features of anoctaminopathy in Saudi Arabia.

Neurosciences (Riyadh) 2015 Apr;20(2):173-7

Department of Neurosciences, MBC 76, King Faisal Specialist Hospital & Research Centre, PO Box 3354, Riyadh 11211, Kingdom of Saudi Arabia. E-mail:

Objective: Characterization of the phenotypic, pathological, radiological, and genetic findings in 2 Saudi Arabian families with anoctaminopathies, and limb girdle muscular dystrophy type 2L (LGMD2L).

Methods: Over a 2-year period from December 2010 to January 2013, the clinical presentations were analyzed and all genes responsible for limb girdle muscular dystrophy (LGMD) were screened in families seen at King Faisal Specialist Hospital and Research Centre, a tertiary care hospital in Riyadh, Saudi Arabia. Out of 66 families with LGMD, we identified 2 families (3.1%) with anoctaminopathy, ANO5 muscular dystrophy.

Results: In the first case, a man presented with asymmetrical calves` muscles weakness and atrophy, which was first noted at age 39. The creatinine kinase (CK) level was >20x normal, muscle biopsy showed necrotizing myopathic changes, and an MRI of the legs showed fatty-tissue replacement to muscle tissue with volume loss involving the gastrocnemius and soleus muscles in an asymmetrical fashion. Minimal disease progression was noted over 18 years of follow up. Exercise induced recurrent rhabdomyolysis was noted over the last 2 years. A novel ANO5 gene mutation (Arg58Trp) was found. In the second family, a male presented at the age of 41 with asymptomatic hyperCkemia and intermittent dyspnea. Over 10 years follow up, he became disabled with muscle cramps, rhabdomyolysis, my oglobinurea, and difficulty ambulating. Muscle biopsy showed necrotizing myopathy and perivascular and interstitial amyloid deposit in skeletal muscle. A homozygous deletion of 11.9 Kb encompassing exon 13 to exon 17 was found in the ANO5 gene. Full cardiac investigations were normal in both patients.

Conclusion: The prevalence of LGMD2L is approximately 3.1% in a Saudi Arabian native LGMD cohort. Slowly progressive, late onset, and asymmetrical weakness was the salient features in these 2 families. The genetic findings were novel and will add to the spectrum of ANO5 known mutations.
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April 2015

Histopathological effects of radiosurgery on a human trigeminal nerve.

Surg Neurol Int 2013 18;4(Suppl 6):S462-7. Epub 2014 Jan 18.

Division of Neurosurgery, Toronto Western Hospital, Faculty of Medicine, Institute of Medical Science, University of Toronto, Toronto, Canada.

Background: Radiosurgery is a well-established treatment modality for medically refractory trigeminal neuralgia. The exact mechanism of pain relief after radiosurgery is not clearly understood. Histopathology examination of the trigeminal nerve in humans after radiosurgery is rarely performed and has produced controversial results.

Case Description: We report on a 45-year-old female who received radiosurgery treatment for trigeminal neuralgia by Cyberknife. A 6-mm portion of the cisternal segment of trigeminal nerve received a dose of 60 Gy. The clinical benefit started 10 days after therapy and continued for 8 months prior to a recurrence of her previous symptoms associated with mild background pain. She underwent microvascular decompression and partial sensory root sectioning. Atrophied trigeminal nerve rootlets were grossly noted intraoperatively under surgical microscope associated with changes in trigeminal nerve color to gray. A biopsy from the inferolateral surface of the nerve proximal to the midcisternal segment showed histological changes in the form of fibrosis and axonal degeneration.

Conclusion: This case study supports the evidence of histological damage of the trigeminal nerve fibers after radiosurgery therapy. Whether or not the presence and degree of nerve damage correlate with the degree of clinical benefit and side effects are not revealed by this study and need to be explored in future studies.
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March 2014

Carotid artery occlusion by rhinoorbitocerebral mucormycosis.

Case Rep Surg 2012 30;2012:812420. Epub 2012 Oct 30.

Division of Neurosurgery, Neurosciences Department, King Faisal Specialist Hospital and Research Center, Riyadh 11211, Saudi Arabia.

Mucormycosis is the third most common invasive fungal infection that particularly occurs in immunocompromised patients. Intracranial and extracranial arteriovenous vasculopathy is a complication that makes this disease more complex and difficult to treat. We describe a 23-year-old female, who presented to her local hospital with acute blindness and diabetic ketoacidosis-induced coma requiring intensive care treatment. She was found to have lesions in the nasal sinuses, orbit, and frontal base. The left carotid artery was occluded from its origin in the neck to the supraclinoid segment and left cavernous sinus involvement. No cerebral infarction was noted. Biopsies obtained by endonasal debridement confirmed mucormycosis. In addition to antimicrobial therapy, she underwent several multidisciplinary approaches to treat her disease. Multiple endonasal, and cranial procedures were done including bilateral orbital exenteration. After prolonged treatment on the intensive care unit she made a remarkable recovery to the point where she was communicating verbally and had normal limb movements and later discharged home. She remained alive and well for two months, but later succumbed to a recurrence of her disease. In conclusion, mucormycosis-induced vasculopathy is a complex problem, which merits aggressive treatment of this invasive disease. It is normally regarded as an indicator of grave prognosis.
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December 2012