A Rare Diagnosis of Aseptic Hypertrophic Pachymeningitis: A Case of Mycobacterial Tuberculosis Origin.

Tubercular meningitis is a rare yet devastating type of extrapulmonary tuberculosis (TB) posing great diagnostic challenges due to the nonspecific clinical presentation of the patients. Here, we present a rare diagnosis of hypertrophic pachymeningitis due to Mycobacterium tuberculosis. A 36-year-old male presented with a history of headaches and giddiness for one month. Neurological examination revealed hypo-reflexive triceps and ankle reflexes. Routine blood tests and autoimmune workup were normal. Brain MRI with contrast revealed diffuse dural thickening, focal leptomeningeal enhancement in the right temporal sulci, and enhancement in both the frontal and parietal convexity and the falx cerebri and along the tentorium cerebelli. Cerebrospinal fluid (CSF) analysis revealed elevated proteins, suggestive of aseptic meningitis. Meningeal biopsy revealed a chronic ill-formed granulomatous inflammatory lesion with occasional acid-fast bacilli, consistent with tubercular pachymeningitis. The patient was administered intravenous (IV) methylprednisolone for five days, following which the symptoms subsided. He was advised tablet prednisolone on discharge, and immunomodulation with rituximab was recommended as outpatient treatment. Hypertrophic pachymeningitis is a rare diagnosis characterized by the inflammation and fibrosis of the dura matter due to a diverse etiology. Tubercular etiology must be considered when the routine laboratory tests are negative, and the diagnosis should be confirmed by meningeal biopsy. The treatment of the underlying cause and corticosteroids remain the mainstay management of hypertrophic pachymeningitis. Hence, mycobacterial tuberculosis should be considered as a possible differential diagnosis while evaluating hypertrophic pachymeningitis, especially when the routine laboratory tests and immunological workup are negative.

Gitelman Syndrome Manifesting With Acute Hypokalemic Paralysis: A Case Report.

Gitelman syndrome (GS) is a rare renal tubulopathy, classically characterized by renal salt wasting and metabolic alkalosis. It is usually an incidental diagnosis, being asymptomatic or with mild symptoms. GS manifesting with acute flaccid paralysis is extremely uncommon. We report a case of GS that mimicked Guillain-Barré syndrome (GBS), manifesting with acute hypokalemic paralysis. A middle-aged male with no known comorbidities presented to our center with paresthesias of all four limbs for one month and progressive, asymmetric limb weakness over the past eight days. Neurological examination revealed hypotonia, global areflexia, and power ranging from 3/5 to 4/5 in all four limbs, leading to our initial clinical diagnosis of GBS. Our patient's laboratory panel revealed hypokalemia, hypomagnesemia, metabolic alkalosis, and hypocalcemia, characteristic of GS. Additionally, he had significantly elevated creatine phosphokinase, suggestive of rhabdomyolysis. Further urine studies revealed renal potassium wasting, confirming the diagnosis of GS. Whole exome genome sequencing for common causative genes and workup for autoimmune disease were both negative. With gradual electrolyte correction, the patient rapidly improved symptomatically. Our case highlights an uncommon initial presentation of GS and emphasizes the need for more literature on its manifestations from the Indian subcontinent.

Dropped Head Syndrome Secondary to Danon Disease: A Case Report.

Dropped head syndrome (DHS) is characterized by neck extensor muscle weakness, which may be isolated or secondary to another neurologic diagnosis. DHS, due to lysosomal storage disorders, has not been reported in the literature. We present a 21-year-old male who had complaints of slowly worsening difficulty swallowing for the past eight years, along with difficulty keeping his head erect. His past medical history was significant for apical hypertrophic cardiomyopathy (HCM), and he had a history of sudden cardiac death in his immediate family. Clinical examination was significant only for neck extensor muscle weakness. His laboratory investigations were unremarkable, save for a significantly elevated creatine kinase (CK). Finally, whole exome sequencing identified a hemizygous stop gain variant in the lysosome-associated membrane protein 2 (LAMP-2) gene, pointing to a diagnosis of Danon disease (DD). DD is a rare, X-linked, inherited disease, due to a defect in the LAMP-2 gene that disrupts lysosomal autophagy. It is characterized by a triad of HCM, skeletal myopathy, and intellectual disability. Males typically suffer a more severe phenotype, and the cardiac disease drives its prognosis. Management involves regular cardiac monitoring, with appropriate physical therapy for myopathy and multidisciplinary treatment for intellectual disability. We suggest that DD be considered in the differential diagnosis for patients with HCM and elevated CK.

Pediatric Posterior Reversible Encephalopathy Syndrome Secondary to Post-Streptococcal Glomerulonephritis: A Case Report.

This case report presents a unique case of a 15-year-old male with post-streptococcal glomerulonephritis (PSGN) who developed posterior reversible encephalopathy syndrome (PRES). The patient presented with symptoms of fever, headache, emesis, visual disturbances, and involuntary movements of all four limbs. On examination, the patient had elevated blood pressure, decreased visual acuity of the left eye, leukocytosis, and uremia. MRI findings showed symmetrical enhancement of superficial and deep watershed areas, predominantly in the occipital and temporal regions. Treatment with antibiotics and anti-hypertensives resulted in the complete resolution of hyperintense lesions seen in brain MRI after three weeks, and the patient remained symptom-free for one month. This case highlights the rare association between PSGN and PRES and emphasizes the importance of monitoring and managing hypertension in patients with PSGN. Understanding the association between these two conditions may lead to earlier diagnosis and treatment of PRES, ultimately improving patient outcomes.

Ipsilateral Hemiparesis in a Patient With Existing Contralateral Hemiparesis: A Case Report of a Rare Presentation of Ischemic Stroke.

Supratentorial strokes causing ipsilateral hemiparesis (ILH) are rare. We report a middle-aged male with multiple atherosclerotic risk factors, who had previously suffered a right-hemispheric stroke that caused left hemiplegia. Subsequently, he presented with worsening left-sided hemiplegia, with imaging revealing a left-hemispheric stroke. Diffusion tensor tract imaging showed crossed motor tracts, with disruption of the left-sided pyramidal tract. During his stay, he developed right hemiplegia due to the expansion of the same left-hemispheric infarct. Potential mechanisms for ILH in a stroke include injury to reorganized tracts following an initial insult and congenitally uncrossed motor tracts. In our patient, after his first stroke, the left hemisphere likely assumed greater ipsilateral motor control, causing ILH after the recent stroke. Our case adds to the literature on this interesting phenomenon and provides further insight into post-stroke recovery.

Idiopathic Intracranial Hypertension following Levothyroxine Replacement Therapy: Systematic Review and a Case Report.

The data on the characteristics of patients with idiopathic intracranial hypertension (IIH) following levothyroxine (LT4) replacement are limited. Here, we report a case and systematically review published cases of idiopathic intracranial hypertension (IIH) following levothyroxine (LT4) replacement. The systematic review was performed as per the PRISMA guidelines. Our patient is a 46-year-old lady with hypothyroidism (thyrotropin: 319 mIU/L, free thyroxine: 0.04 ng/dl), treated with 100 µg.d of LT4 and presented a month later with headache, visual diminution, bilateral lateral rectus palsies, and papilledema. Cerebrospinal fluid (CSF) pressure was 32 cmH2O. Drainage of CSF, oral acetazolamide, and modification of LT4 dose resulted in prompt symptomatic improvement and complete reversal of IIH. In the systematic review (n = 21), the median age of patients (7 males) was 13 (IQR: 8.8- 26.5) years. The median duration of hypothyroid symptoms was 4 (n = 10, IQR: 0.44-6.25) years whereas that from initiation of LT4 replacement to the diagnosis of IIH was 2 (n = 20, IQR: 1.17-4) months. Initial median serum thyrotropin and thyroxine were 100 (n = 14, IQR: 72.5-421.6) mIU/L, and 1.13 (n = 12, IQR: 1.0-2.45) µg/dl which changed to 2.2 (n = 7; IQR: 0.23-3.40) mIU/L and 8.90 µg/dl (n = 8, IQR: 6.43-14.85 µg/dl), respectively at diagnosis of IIH after LT4 treatment with median daily LT4 doses of 0.89 (n = 8, IQR: 0.60 - 1.17) times the maximum recommended dose for age. To conclude, we report an adult woman with IIH following LT4 replacement for primary hypothyroidism, a rare entity. Pediatric age, prolonged symptom duration, and use of higher LT4 replacement dose may be associated with IIH following LT4 replacement.

Cardioembolic Stroke in Young: A Case of Atrial Myxoma Origin.

Stroke is one of the leading causes of mortality and disability. It can be rarely caused by cardiac myxoma. Sometimes stroke may be its first clinical manifestation. Here we report a case of posterior circulation stroke in left atrial myxoma. A 45-year-old female patient presented with a history of recurrent episodes of dizziness and headache of three months duration. Neurological examination showed impaired tandem gait. Magnetic resonance imaging (MRI) of the brain revealed infarction in the left posteroinferior cerebellar hemisphere. Echocardiography of the patient revealed a large left atrial mass suggestive of atrial myxoma and an ejection fraction of 60%. The patient was operated on for atrial myxoma two days after the diagnosis, and histopathology confirmed the diagnosis. Postoperatively she remained well and was managed on anti-platelet drugs. Atrial myxoma should be considered as a possible differential while evaluating a case of cardioembolic stroke, and echocardiography detects the presence of an atrial myxoma. It is also essential that atrial myxomas are managed early to prevent recurrent strokes.

Evaluation of Acute Symptomatic Seizures and Etiological Factors in a Tertiary Care Hospital From a Developing Country.

INTRODUCTION: The etiologies of acute symptomatic seizures (ASS) differ across the globe. We aimed to evaluate the etiological spectrum of acute seizures and to observe the pattern of seizure types among study participants. METHODOLOGY: We conducted this prospective study from 2016 to 18. We included all patients aged 20 years or older, presenting with ASS. We excluded those with pseudoseizures. We performed appropriate descriptive analyses to describe the demographic details, etiology of ASS, and pattern of ASS. RESULTS: One hundred and thirty-eight patients were enrolled, constituting about 0.8% of total hospital admissions. The mean age at presentation was 44.33 ± 17.73 years. The most common etiologies for ASS were cerebrovascular accidents (CVA - 32.6%), neuroinfections (26.8%), metabolic derangements (13%), alcohol withdrawal (10.9%), and intracranial tumors (4.3%). 71% of the patients presented with only a single episode of ASS. The predominant type of seizure was generalized tonic-clonic seizures, seen in 70.2% of all patients, followed by focal with the bilateral tonic-clonic type (15.9%) and focal seizures (10.1%). New-onset seizures presenting as status epilepticus were observed in 3.6%. DISCUSSION: CVA and neuroinfections were the most common causes of ASS in our study, highlighting the need for community awareness of these conditions and the need to seek rapid care. The majority of our patients had only a single episode of seizures, with generalized seizures being the most common type, followed by focal onset seizures.

Transient Ischemic Attack Due to Unruptured Basilar Artery Aneurysm.

Intracranial aneurysms are typically asymptomatic. They are usually incidentally detected or detected only after rupture. Ischemic stroke or transient ischemic attack (TIA) due to unruptured intracranial aneurysms (UIAs) is rare. A 79-year-old male with well-controlled hypertension and hypothyroidism, presented with complaints of sudden-onset weakness of the right upper limb and lower limb, followed by altered sensorium and a fall. Two hours later, he had fully recovered. Neurologic examination was unremarkable. Computed tomography of the brain revealed a dilated and tortuous basilar artery, suggestive of an aneurysm compressing the left midbrain and pons, with no evidence of intracranial bleed. Further, magnetic resonance imaging with an angiogram revealed multiple lacunar infarcts in the posterior circulation, distal to the aneurysm. Finally, a cerebral angiogram confirmed a partially thrombosed, fusosaccular aneurysm, arising from the left vertebral and basilar arteries. In view of frailty and long vessel segment involvement, surgery was not advised. He was treated medically, with appropriate antiplatelets and prophylactic antiepileptics. On follow-up, he had no neurologic deficit and had suffered no later ischemic or hemorrhagic events. UIAs may cause brainstem strokes via thrombosis of the parent vessel, emboli from the thrombus, or compression of the parent artery. In our case, compression, the least common mechanism, appears to have caused the TIA, with emboli potentially responsible for the silent lacunar infarcts. Fusiform aneurysms of the vertebrobasilar system have a poor natural history. In elderly patients presenting with ischemic events due to UIAs of the vertebrobasilar system, surgical intervention can be risky. So, medical treatment with antiplatelets is recommended. UIAs should be considered in the differential diagnosis of patients with TIAs, and such patients should have a visualization of intracranial arteries.

Hemicrania continua secondary to neurogenic paravertebral tumor- a case report.

OBJECTIVES: Hemicrania continua (HC) is one of the trigeminal autonomic cephalalgias (TAC), where sympathetic dysfunction and autonomic dysfunction resulting in parasympathetic over activation with some evidence of sympathetic inhibition have been suggested as probable causes. However, cases of hemicrania continua secondary to sympathetic dysfunction due to neurogenic paravertebral tumor impinging on the sympathetic chain has not been previously reported. In this case, the probability of the sympathetic dysfunction was more likely based on the clinical features and management. CASE PRESENTATION: A 23-year-old female presented with a chief complaint of right unilateral pain in the retro-bulbar, head and facial region for the past three years. An initial MRI of the brain was negative, whereas an MRI of the spine was advised to rule out a cervicogenic origin of the pain. The MRI revealed a well-defined mass lesion within right paravertebral region at T3 indicative of a neurogenic tumor. The patient was diagnosed with probable hemicrania continua secondary to neurogenic tumor impinging on adjacent sympathetic chain. A trial of indomethacin 75 mg/day was advised, which provided complete relief of the headache. The patient was referred to a neurologist for management of the neurogenic tumor. CONCLUSIONS: Headache disorders may be secondary to pathologies and comprehensive evaluation and accurate diagnosis are essential. Knowledge of neuroanatomy is paramount to understand and explain underlying pathophysiological mechanisms. Multidisciplinary management is essential in complex orofacial cases.