Botulinum toxin type A therapy in a patient with cervical dystonia and amyotrophic lateral sclerosis.
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Biomedical subjects
Publications and source records attributed to Ashok Verma.
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Sialorrhea is frequently a socially disabling symptom in patients with bulbar amyotrophic lateral sclerosis (ALS). In this open-label prospective study, we report the effect of botulinum toxin A (Botox) injection into the parotid glands in 10 patients with bulbar ALS and socially disabling sialorrhea. We applied three different outcome measures to determine the effect of Botox therapy on sialorrhea. Botox significantly improved the degree of sialorrhea and a drooling impact score and, by inference, the quality of living, in over half of the patients with bulbar ALS and severe sialorrhea. The beneficial effect of Botox lasted for at least 2 months in those who responded. No major adverse effects were noted. Local injection of a small dose of Botox into the parotid glands can control sialorrhea and potentially improve living quality in some patients with bulbar ALS.
Isolated cerebral mucormycosis is a rare but life-threatening infection that generally occurs in patients with intravenous drug abuse or immune deficiency. We report a case of primary cerebral mucormycosis in a healthy adult. Whole body autopsy in this case revealed cerebral mucormycosis with prominent vascular pathology and hemorrhagic necrosis. No nasal sinus, orbital or other primary locus of fungus infection was discovered. Review of the previously reported 30 cases of isolated cerebral mucormycosis revealed associated systemic predisposition in 11 patients and history of intravenous drug abuse in 17 cases. In the remaining two cases, the diagnosis of fungal infection was made only after surgical exploration. Early tissue diagnosis and the consequent surgical excision of the necrotic tissue and aggressive antifungal therapy might salvage life in this fatal condition.
BACKGROUND: A viral etiology of amyotrophic lateral sclerosis (ALS) has been proposed because of the selective vulnerability of motor neurons to certain viruses. During the last 20 years, at least 19 cases of ALS or ALS-like disease have been reported in HIV-1 (HIV) seropositive individuals. OBJECTIVE: To describe two cases of clinically definite ALS in patients with HIV infection and to review the previously reported cases of HIV-associated ALS syndrome. SETTING: A multidisciplinary ALS center and Neuro-AIDS clinic at a tertiary care university hospital. PATIENTS/DESIGN: We investigated and prospectively monitored two patients who had developed clinically definite ALS by El Escorial criteria several years after acquiring the HIV infection. The previously reported cases of ALS or ALS-like disease in patients with HIV infection were reviewed for comparison and contrast with the characteristics of sporadic ALS. RESULTS: The clinical course of ALS in our two HIV seropositive individuals mirrored that of classical sporadic ALS. A review of previously described 19 patients with ALS syndrome revealed clinically definite ALS in 4 cases and clinically probable or possible ALS in 15. ALS commenced at different stages of the HIV disease; in 7 patients, HIV infection was discovered contemporaneously with diagnosis of ALS. CD4+ T cell count ranged from 2 to 560 cells/mm3. Three (1 definite ALS) of the fatal cases were studied at autopsy and all exhibited pathology outside the motor neuron pool. Unlike our patients, 7 of 8 patients with HIV-associated ALS syndrome receiving HAART demonstrated at least partial recovery of their motor deficit. CONCLUSIONS: ALS-like syndrome can occur in association with HIV infection; however, the causal relationship remains uncertain. Patients with ALS syndrome related to HIV infection are generally younger in age and often demonstrate pathology outside the motor neuron system. Patients with HIV-associated ALS syndrome may improve following antiretroviral therapy. An aggressive HAART regimen to reduce viral load should be pursued in all such cases.
The nosologic boundary of neuropathic scapuloperoneal syndrome (Davidenkow's syndrome) remains ill defined and its genetic basis is unknown. A case of Davidenkow's syndrome with the monochromosomic 17p11.2 deletion that often is associated with hereditary neuropathy with liability to pressure palsies (HNPP) is described. The other allele at chromosome 17p11.2 locus was of normal length, and direct sequencing of the coding region of the peripheral nerve protein-22 gene in this allele revealed no additional mutation. The deleted allele in the proband was inherited from the paternal line in which the affected members had a late onset Charcot-Marie-Tooth type 1 clinical phenotype. This observation suggests that the rare Davidenkow's syndrome is clinically related to HNPP and its genotype could be a chromosome 17p11.2 deletion.
OBJECTIVE: The goal of the present study was to find out if recurrence can be predicted in cases of allergic fungal sinusitis. We also studied the influence of postoperative corticosteroid therapy on recurrence following surgery. STUDY DESIGN AND SETTING: This study was conducted at the ENT Department of Al Nahdha Hospital, which is a tertiary referral and teaching hospital in Muscat, Sultanate of Oman. The study is a retrospective analysis of 32 cases of allergic fungal sinusitis. Age, sex, extent of disease, and preoperative serum IgE levels were compared in patients who had recurrence with those who did not. We also studied the incidence, onset, and severity of recurrence in patients who received systemic corticosteroid as postoperative therapy and compared these values to those who received nasal corticosteroid spray only. RESULTS: No statistically significant difference was noted in the parameters of age, sex, extent of disease, and preoperative serum IgE levels when these values were compared in the group of patients who had recurrence (8 patients) with the group of patients who did not (32 patients). No statistically significant difference was found in the incidence of recurrence in patients in whom systemic corticosteroids were used postoperatively (17 patients) compared with patients who used nasal corticosteroid spray only (15 patients). However, when the patient had a recurrence, when it occurred it was earlier and more severe in patients who used nasal corticosteroid spray only (4 patients). CONCLUSIONS: At the present time, it is not possible to predict recurrence using parameters of age, sex, extent of disease, and serum IgE levels. Larger number of patients preferably in a prospective multicenter meta study are required to address this issue. Though use of systemic corticosteroid does not decrease the incidence of recurrence, it may delay the onset of recurrence and decrease the severity of recurrence.
OBJECTIVE AND IMPORTANCE: Intraventricular hemorrhage (IVH) is known to cause acute obstructive hydrocephalus, refractory elevated intracranial pressures (ICPs), and lowered cerebral perfusion pressures, leading to cortical ischemia. Frequent obstruction of external ventricular drains as a result of thrombus is a recurring theme. We present a case of IVH secondary to periventricular arteriovenous malformation (AVM) that was not visible at admission angiography and was treated by intraventricular infusion of recombinant tissue plasminogen activator before surgical intervention. CLINICAL PRESENTATION: An 11-year-old boy presented with acute onset of headache followed by two seizures, loss of consciousness, decerebration, right temporal hematoma, IVH, and acute obstructive hydrocephalus. INTERVENTION: A right external ventricular drain was placed but functioned poorly. ICP could not be controlled by conventional methods. Five milligrams of recombinant tissue plasminogen activator was injected into the ventricular system via the external ventricular drain. This was repeated daily for 4 days. This treatment resulted in progressive improvement in ICP and clinical status. Once the clot partially cleared, magnetic resonance imaging and magnetic resonance angiography suggested the presence of a right periventricular arteriovenous malformation, which was confirmed by angiography and subsequently resected. CONCLUSION: Recombinant tissue plasminogen activator is effective in resolving IVH causing obstructive hydrocephalus and uncontrollable ICP posing a life-threatening situation, secondary to ruptured arteriovenous malformation, before surgical intervention.
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Giant cell granuloma of the skull base is a distinct rare clinicopathologic lesion, which progressively destroys the involved bone. It causes increased intracranial pressure and mass effect on the brain. Histologically it may mimic an aneurysmal bone cyst, giant cell tumour and the brown tumour of hyperparathyroidism. Although cited by some authors, the role of trauma in its aetiology is still considered controversial. Authors present an interesting case of a 12 year old Omani boy who sustained a cricket bat injury to his right temporal region from which he initially recovered but later on progressively developed vertigo, tinnitus, right hearing loss and a mild right facial weakness. Computed tomography (CT scan) and Magnetic resonance imaging (MR scan) revealed a large destructive lesion of the temporal bone. Microsurgical excision was curative. Interesting clinicoradiological findings are presented with a brief review of the literature.