Case Reports in Neurological Medicine

Spontaneous intracranial hypotension (SIH) is a rare condition caused by a cerebrospinal fluid (CSF) leak. It is diagnosed by clinical features that include an orthostatic headache combined with imaging findings demonstrating intracranial hypotension and a CSF leak. We present the case of a 45-year-old woman with an orthostatic headache who was found to have a sagging brain with a downward-displaced cerebellum and pachymeningeal enhancement with gadolinium contrast. This was initially misidentified as a Chiari I malformation, but the constellation of symptoms and MRI findings were later recognized as characteristic of SIH...

A link between intractable hiccups, as the initial symptom, and a possible neuromyelitis optica spectrum disorder (NMOSD) diagnosis is confusing but vital and may not be made by health care providers (HCPs) if they are not aware of the 2015 NMOSD criteria. Early diagnosis and adequate treatment are essential to prevent disease progression. We report the case of a 46-year-old Puerto Rican female who presented intractable hiccups when she was 31 (in 2004). Almost 15 years passed since the initial symptom, and after two severe relapses, she received a formal NMOSD diagnosis in March 2019...

A 51-year-old man with known Leber's hereditary optic neuropathy (LHON) presented with worsening lower extremity weakness and numbness. Following an episode of myelopathy two years before, he had been ambulating with a walker but over two weeks became wheelchair bound. He also developed a sensory level below the T4 dermatome to light touch, pinprick, and vibration. MRI of his cervical and thoracic spine showed a nonenhancing T2 hyperintense lesion extending from C2 to T12. At his presentation two years earlier, he was found to have a longitudinally extensive myelopathy attributed to his LHON...

Tumoral calcinosis involves focal calcium deposits in the soft tissues surrounding a joint and most commonly occurs in the hips and elbows, rarely in the cervical spine. Furthermore, it has not been known to be associated with pathologic fractures. To the best of our knowledge, our case report highlights the first case of a pathologic type II odontoid fracture associated with adjacent tumoral calcinosis, resulting in pain, dysphagia, and severe spinal stenosis. The patient underwent a posterior occipitocervical fusion and C1 laminectomy, along with planned tracheostomy and gastrostomy to avoid expected difficulty with postoperative extubation and dysphagia...

Optic neuritis is a rare demyelinating disorder, which involves the optic nerve. It can be a monophasic self-limiting illness due to postinfectious or postvaccination etiology. It can also be an initial presentation of a relapsing demyelinating disorder such as multiple sclerosis or neuromyelitis optica spectrum of disorders. It is characterized to aquaporin-4 antibody-rich areas in the brain, optic nerve, and spinal cord. The hypothalamus and periventricular area are also rich in specific antibodies and may lead to dysfunction in the hypothalamic-pituitary axis...

Antiepileptics drugs are the mainstay of the management of epilepsy in children. Sodium valproate (VPA) and carbamazepine (CBZ) are widely used medications in childhood epilepsy. Hyperammonemia has been described as a known side effect of valproate therapy. It is known that VPA-associated HA is common among patients who hold genetic mutations of the carbomoyl phosphatase synthase 1 gene (CPS1). Aggravation of self-limited epilepsy with centrotemporal spikes (SLECTS) is a rare side effect of CBZ. Here, we present a child who had CBZ-induced aggravation of rolandic epilepsy and VPA-induced HA encephalopathy in the background of an unrecognised heterozygous gene variant of CPS1...

Myasthenia gravis is a neuromuscular autoimmune disease that results in skeletal muscle weakness that worsens after periods of activity and improves after rest. Myasthenia gravis means "grave (serious), muscle weakness." Although not completely curable, it can be managed well with a relatively high quality of life and expectancy. In myasthenia gravis, antibodies against the acetylcholine receptors at the neuromuscular junction interfere with regular muscular contraction. Although most commonly caused by antibodies to the acetylcholine receptor, antibodies against MuSK (muscle-specific kinase) protein can also weaken transmission at the neuromuscular junction...

Stab wounds to the cervical spine are less common than injuries from road accidents, sports injuries, and falls. The presence of vital, vascular, neural, respiratory, and digestive structures in the neck region mean that this kind of spinal injury is generally critical, and its management is a challenge. We report a unique case of a previously healthy 17-year-old adolescent admitted for quadriplegia secondary to a stab wound to the cervical spine at the C4C5 level. There was no surgical indication. The patient underwent physiotherapy...

Compressive tibial mononeuropathies are uncommon and can be caused by conditions including posterior compartment syndrome, soleal sling syndrome, and tarsal tunnel syndrome. Therefore, it is critical to consider noncompressive etiologies when a tibial mononeuropathy is suspected. This is a patient with a history of rare inherited immune dysregulation that presented to the electrodiagnostic laboratory with severe neuropathic pain in the right foot associated with plantarflexion weakness, concerning for a tibial mononeuropathy...

BACKGROUND: Lemierre's syndrome is a rare condition of internal jugular vein thrombosis following oropharyngeal infection. While it usually results from Fusobacterium necrophorum infection, atypical cases associated with other pathogens have been reported. OBJECTIVE: To describe a unique case of pediatric Lemierre's syndrome with Streptococcus viridans infection resulting in cavernous sinus thrombosis and oculomotor, trochlear, and abducens nerve palsies. Case Report ...

Paraplegia after spine surgery is a catastrophic complication. Here, we present a patient who, following laminectomy and fusion for decompression of metastatic tumor, developed paraplegia. We tried to find out the possible reason for the paraplegia. Due to prolonged hypotension during operation and new onset of pneumothorax, we think that intraoperative prolonged hypotension leads to the spinal cord ischemia which may cause neurological deterioration of paraplegia. Maintaining hemodynamic stability during spinal surgery is very important...

Introduction: Cerebral air embolism is a rare, yet serious neurological occurrence with unclear incidence and prevalence. Here, we present a case of fatal cerebral arterial and venous cerebral gas embolism in a patient with infective endocarditis and known large right-to-left shunt and severe tricuspid regurgitation following pressurized fluid bolus administration. Case Presentation . A 32-year-old female was admitted to the medical intensive care unit from a long-term acute care facility with acute on chronic respiratory failure...

Multiple sclerosis (MS) is an immune-mediated demyelinating disorder involving the central nervous system (CNS). It is common amongst young females. Although the exact cause of MS is yet unknown, viral infections such as EBV, environmental factors, and autoimmune and genetic mechanisms involving HLA-DRB1 loci are implicated. Familial MS is reported from some geographic locations and ethnic groups but is thought to be rare in Asia. In this paper, we present both a Sri Lankan mother and her son, with clinically definite MS conforming to McDonald's 2017 clinical and MAGNIMS 2016 radiological criteria...

We present a case report of a patient with incomplete Susac syndrome. He had cognitive impairment, corpus callosum lesions, and vestibulocochlear dysfunction on brainstem auditory evoked responses. He was treated with methylprednisolone and plasmapheresis, improved, and then, also received rituximab. His improvement has been lasting as of this writing. This case shares our experience with a successful treatment of this rare condition that is incompletely understood and lacks well-established treatment guidelines...

OBJECTIVE: This report is the first one to describe the possibility to use "self-administered" photobiomodulation (PBM) for Bell's palsy (BP) treatment. BACKGROUND: BP is a peripheral disorder of the facial nerve causing sudden paralysis of unilateral facial muscles, and PBM has been successfully suggested for its treatment without any side effect. This is the first case report where a laser device was successfully used at home by the patient herself to treat BP opening new perspectives on the therapy of this disease...

Neuromyelitis optica spectrum disorder (NMOSD) is an immune-mediated inflammatory condition involving spinal cord and optic nerves. Diagnosis of NMOSD is done by aquaporin-4 antibody (AQP4) in patients with optic neuritis. Myelin oligodendrocyte glycoprotein (MOG) expressed on the oligodendrocyte cell surface and on the outermost cell surface of the myelin sheath may also be present in patients with NMOSD bilateral optic neuritis. Here, we describe a case of a thirty-nine-year-old-female with recurrent bilateral optic neuritis with positive anti-MOG antibody, and anti-MOG syndrome has not previously been reported from Nepal...

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...

CACNA1A encodes the pore-forming α 1 subunit of the neuronal voltage-gated Cav2.1 (P/Q-type) channels, which are predominantly localized at the presynaptic terminals of the brain and cerebellar neurons and play an important role in controlling neurotransmitter release. Mutations in CACNA1A have been associated with several autosomal dominant neurologic disorders, including familial hemiplegic migraine type 1, episodic ataxia type 2 (EA2), and spinocerebellar ataxia type 6. A 37-year-old woman presented with a history of slowly progressive, activity-induced stiffness, and pain in her right leg since age 15 and cervical dystonia since age 20...

Dermoid cysts account for only a small fraction of intracranial masses, with extradural dermoid cysts being considered a much rarer entity than those located intradurally. Intracranial dermoid cysts vary in clinical presentations: some maybe asymptomatic whilst others harbor features of raised intracranial pressure, neurological deficits, or even aseptic meningitis. Dermoid cysts may also present with cutaneous lesions. Herein, we report a rare case of a 1-year-old female presenting with a midline, scalp abscess...

Invasive infection with Lancefield group C streptococci in humans is extremely rare, with the vast majority of clinical isolates belonging to Streptococcus dysgalactiae subsp. equisimilis . We report a case of meningoencephalitis in a 69-year-old man caused by Streptococcus equi subsp. equi, a microbe that causes strangles in Equus caballus (i.e., the horse). This is only the fourth infection with this subtype of the central nervous system (CNS) reported in humans. The invasiveness of these bacteria, known to be capable of releasing strongly immunogenic exotoxins, is illustrated by white matter lesions that are present in the acute phase...