Hemispherectomy for dominant hemisphere Rasmussen's Encephalitis - how late is too late?

It is unclear whether a dominant hemispherectomy/hemispherotomy in someone with Rasmussen's Encephalitis (RE) may produce a satisfactory outcome when performed over the age of 40 years. Important questions include whether RE may continue to evolve three decades after onset, and whether a hemispherectomy may adequately shift language function when performed in older ages. Two cases illustrate seizure, language, motor and functional outcomes after dominant hemispherotomies. The cases were selected from an epilepsy surgery database of procedures performed at a private hospital in Cape Town, South Africa, spanning the period 1998-2023. A man in his 40s with epilepsy since childhood and dominant hemisphere RE partially regained impaired comprehension and ambulation, while expressive language function did not recover post-hemispherotomy. By contrast, a young teenage patient with dominant hemisphere RE demonstrated considerable recovery of expressive and receptive language and ambulation post-surgery. Both remain seizure-free. These two cases demonstrate that a dominant hemispherotomy, when performed on a quadragenarian, may produce a satisfactory, albeit inferior, functional outcome in comparison to when performed in childhood. RE may cause progressive neurological dysfunction in the late thirties and older and should be considered in patients presenting with functional decline decades after disease onset.

Tumor necrosis factor-alpha antagonists in patients with complicated spinal tuberculosis: A case series and literature review.

INTRODUCTION: Spinal tuberculosis is often associated with poor outcomes; host-directed inflammation involving the spine contributes to this disability. METHODS: A retrospective review of patients with complicated spinal tuberculosis having received tumor necrosis factor-alpha (TNF-α) antagonists at a referral hospital in South Africa. A literature review was performed to identify all published cases of complicated spinal tuberculosis that received a TNF-α antagonist as part of their treatment. RESULTS: We describe 23 cases, of which 19 were previously reported in the literature. All patients were treated with either thalidomide (n=6) or infliximab (n=16), except for one who received both. All in all, 21 (91%) cases improved neurologically and, at the end of follow-up, 18 could walk. CONCLUSION: There is accumulating experience to confer the efficacy and safety of TNF-α antagonists in treating complicated spinal tuberculosis cases. Evidence from randomized controlled trials is urgently required to substantiate these findings.

The Epidemiology and Phenotypes of Ocular Manifestations in Childhood and Juvenile Myasthenia Gravis: A Review.

Myasthenia gravis (MG) appears to have a similar incidence among adult populations worldwide. However, epidemiological and phenotypic differences have been noted among children and juveniles with MG. We reviewed the literature on childhood- and juvenile-onset MG among different populations, with the focus on ocular involvement, antibody profiles, the genetic susceptibility to juvenile MG phenotypes, the use of immune treatments, and the reported responses of extraocular muscles to therapies. Although epidemiological studies used different methodologies, reports from Asia, compared to Europe, showed more than two-fold higher proportions of prepubertal onset (before 12 years) vs. postpubertal-onset juveniles with MG. Compared to European children, ocular MG was 4-fold more frequent among Asian children, and 2-3-fold more frequent among children with African ancestry both in prepubertal and postpubertal ages at onset. These results suggest genetic influences. In Asia, HLA-B * 46 and DRB1 * 09 appeared overrepresented in children with ocular MG. In Europe, children with MG had a significantly higher rate of transforming from ocular to generalized disease and with an overrepresentation of HLADRB1 * 04. Although treatment regimens vary widely and the responses to immune therapies of the ocular muscles involved in MG were generally poorly described, there were indications that earlier use of steroid therapy may have better outcomes. Reports of treatment-resistant ophthalmoplegia may be more frequent in African and Asian juvenile MG cohorts compared to Europeans. Genetic and muscle gene expression studies point to dysregulated muscle atrophy signaling and mitochondrial metabolism pathways as pathogenetic mechanisms underpinning treatment-resistant ophthalmoplegia in susceptible individuals. In conclusion, phenotypic differences in juveniles with ocular manifestations of MG were evident in different populations suggesting pathogenetic influences. Treatment responses in MG-associated ocular disease should attract more careful descriptive reports. In MG, extraocular muscles may be vulnerable to critical periods of poor force generation and certain individuals may be particularly susceptible to developing treatment-resistant ophthalmoplegia. The development of prognostic biomarkers to identify these susceptible individuals is an unmet need.

Anterior temporal lobectomy: A cross-sectional observational study of potential surgical candidates at a single institute.

BACKGROUND: Epilepsy is a common neurological disorder, associated with serious cognitive, physical, and psychosocial burdens. Mesial temporal lobe epilepsy (mTLE) is the commonest form of focal epilepsy. The aim of this study was to establish the incidence of patients with electroencephalographic epileptiform discharges consistent with mTLE attending a tertiary hospital in South Africa, and determine whether these patients may be candidates for anterior temporal lobectomy. METHODS: This was a cross-sectional observational study of all patients receiving scalp electroencephalograms (EEG) performed at the Groote Schuur Hospital Neurophysiology laboratory during the period January 1, 2017-December 31, 2019. Where magnetic resonance imaging (MRI) brain scans had been performed, these were assessed for corroborative evidence of mTLE. RESULTS: Over the 3-year period, 4 342 EEGs were assessed. A total of 411 (11%) showed epileptiform discharges consistent with all epilepsy types. Of these, 327 (69%) were of focal onset and 108 (33% of all focal onset epilepsies) were consistent with mTLE. Of the patients with electroencephalographic features of mTLE, only 27 (25%) had had MRI brain scans performed according to an epilepsy surgery protocol. None of these patients had been considered for surgery. CONCLUSION: Surgery, especially anterior temporal lobectomy, is widely acknowledged to be an efficacious and cost-effective intervention in patients with drug-resistant mTLE. The findings of our study suggest that patients with mTLE in our setting are under-investigated for potential surgery; and that it is under-utilized. These findings are in line with similar studies in both well-resourced and resource-constrained countries. Our study also highlights the utility of EEG as a practical screening tool to identify potential surgical candidates, as well as the establishment of an EEG and MRI database to assist in recognizing these patients.

Recurrent Hodgkin's Disease Presenting as a Clinically Isolated Cavernous Sinus Syndrome.

BACKGROUND: Hodgkin's disease involving the central nervous system is uncommon and is usually seen in patients with uncontrolled systemic disease or those who have had multiple episodes of recurrent disease. Common symptoms of intracranial Hodgkin's disease are motor and/or sensory deficits, headache, papilloedema, coma, and seizures. The rarity of Hodgkin's disease presenting with intracranial involvement is marked, but patients presenting with cavernous sinus syndrome is even rarer. Despite its rarity, the presence of a cavernous sinus syndrome in a patient with a known history of Hodgkin's disease warrants full utilization of modern diagnostic techniques in terms of investigation. Case Presentation. A 34-year-old woman, known with previous Hodgkin's lymphoma and now in remission for the past 7 years, presented with signs and symptoms suggestive of a left cavernous sinus syndrome. She was otherwise systemically well with no other complaints. Extensive investigations revealed no obvious cause for the cavernous sinus syndrome. A CT chest revealed subclinical axillary lymphadenopathy, which on excisional biopsy confirmed recurrent Hodgkin's disease. The patient's sole clinical presentation of her recurrent disease was the cavernous sinus syndrome, with no other clinically obvious systemic signs or symptoms to suggest a relapse. This was treated with steroids, and clinical improvement was noted; she was referred to oncology for extensive chemotherapy. CONCLUSIONS: Whilst there is a paucity of literature around this topic, it must be remembered by the clinician that cavernous sinus syndrome may be the sole clinical presentation of recurrent Hodgkin's disease. Even though it is reported that lymphomatous involvement in the brain usually occurs late in the disease process, this case is evidence that cavernous sinus syndrome may precede other systemic signs and symptoms. Recognising this possibility will ensure a rigorous search for Hodgkin's disease, an early and effective diagnosis, and a better prognosis in affected patients.

Established association of legionella with rhabdomyolysis and renal failure: A review of the literature.

Legionella causes 2-15% of community acquired pneumonia cases that require hospitalization and it is the second most common cause of serious pneumonia that needs admission in an intensive care unit. Since the first published case in 1980, there are a further 22 published case reports on the direct correlation between rhabdomyolysis, renal failure and Legionnaires' disease. All but two patients survived with antibiotics and dialysis. Clinicians should be cognisant of this established triad and correlation of Legionnaires' disease, renal failure and rhabdomyolysis, as failure to do so and initiate treatment early has proven to increase mortality significantly in affected patients.

A CD99+ primary spinal central PNET in a pregnant patient- therapeutic concerns and issues.

Spinal tumours in pregnancy are rare. Spinal tumours account for only 10-15% of all primary central nervous system (CNS) tumours. Most spinal tumours in pregnant women have been described as meningiomas or vascular tumours. We present the unique case of a pregnant patient presenting with a CD 99+ primary spinal central PNET.