Asymptomatic idiopathic intracranial hypertension post female to male gender transition

A 23- year-man post female to male (FTM) gender transition was found to have bilateral papilloedema at a routine optician visit. The patient was referred on for formal ophthalmological and neurological assessments. Optical coherence tomography (OCT) confirmed the presence of bilateral papilloedema. The patient was entirely asymptomatic and had no medical history. He took testosterone intramuscularly once per month. Neurological examination was otherwise normal. Investigations including routine blood panels, CT brain, MRI brain and cerebral MR venogram were all normal. Lumbar puncture yielded cerebrospinal fluid (CSF) normal in appearance but demonstrated raised intracranial pressure. In the absence of other causative aetiologies a diagnosis of idiopathic intracranial hypertension (IIH) was made. Treatment was commenced with acetazolamide and the patient was discharged with outpatient ophthalmological and neurological follow-up.

Upward gaze palsy and convergence insufficiency as a rare presentation of primary intraventricular haemorrhage

A primary intraventricular haemorrhage (PIVH) usually presents with non-localised neurological symptoms since the haematoma is limited to the ventricles. However, it is sometimes associated with focal neurological signs, whose pathophysiologies are not confirmed. Here, we report on a case of PIVH who showed rare manifestations in the acute stage: upward gaze palsy and convergence insufficiency. The CT and MRI showed intraventricular haematoma without evidence of parenchymal haemorrhage, local mass effect around midbrain or hydrocephalus. There had been bilateral papilloedema, and it resolved along with improvement of the ophthalmic symptoms, suggesting a possible causal relation to increased intracranial pressure. The ophthalmic abnormalities suggested injury of the rostral part of the midbrain, especially the region around the dorsal midbrain tectum. It should be known that PIVH is one of the causes of acutely developing upward gaze palsy and convergence insufficiency.

Malignant idiopathic intracranial hypertension revealed a hidden primary spinal leptomeningeal medulloblastoma

Frequently the cause of raised intracranial pressure remains unresolved and rarely is related to spinal tumours, moreover less to spinal medulloblastoma without primary brain focus. An 18-year-old woman had a 3-month history of headache and impaired vision. Neurological examination revealed bilateral sixth cranial nerve palsies with bilateral papilloedema of grade III. No focal brain or spine lesion was found on imaging. Consecutive lumbar punctures showed high opening pressure and subsequent increasing protein level. Meningeal biopsy was negative. At one point, she developed an increasing headache, vomiting and back pain. Spine MRI showed diffuse nodular leptomeningeal enhancement with the largest nodule at T6–T7. Malignant cells were detected in cerebrospinal fluid. She underwent laminectomy with excisional biopsy, and pathology showed medulloblastoma WHO grade IV. She was treated with chemotherapy and craniospinal irradiation and made a good recovery.

CRANIAL ACTINOMYCOSIS AND SEPTIC CEREBRAL VENOUS THROMBOSIS

60 years old female diagnosed to be having CNS actinomycosis 3 years back presented with neurovisual loss for which she was treated with penicillin. Her vision improved and she was discharged. Now she presented with severe headache in the occipital region of 3 months duration. On general examination she was conscious and alert. On CNS examination she had bilateral papilloedema with secondary optic atrophy. Her motor, sensory and cerebellar functions were normal.

Rare Case of Unilateral Disc Oedema Secondary to Idiopathic Intracranial Hypertension

Majority of idiopathic intracranial hypertension (IIH) cases present with bilateral papilloedema. Unilateral papilloedema is a rare condition in IIH. This is a report of one such unusual case. Papilloedema is clinically defined as swelling of the optic nerve head secondary to raised intracranial pressure.1 Disc oedema is a non-specific term that includes papilloedema but also refers to a disc swollen from other causes. Classically, presence of papilloedema is most commonly due to an intracranial space occupying lesion leading to raised intracranial pressure; however, it may be caused due to other aetiologies such as hydrocephalus, shunt failures, idiopathic intracranial hypertension and drugs.2 Idiopathic intracranial hypertension, also known as pseudotumour cerebri, is a disorder characterised by increased intracranial pressure of unclear pathogenesis in the absence of other structural and obstructive lesions. This is a case of acute established unilateral disc oedema secondary to idiopathic intracranial hypertension.

A case report on Syndrome of transient Headache and Neurological Deficits with cerebrospinal fluid Lymphocytosis (HaNDL)

HaNDL syndrome (transient headache and neurological deficits with cerebrospinal fluid lymphocytosis) is a rare headache disorder previously known as Migraine with cerebrospinal fluid pleocytosis. The disease is characterized by one or more episodes of headache and transient neurological deficits associated with cerebrospinal fluid lymphocytosis. It is a benign disorder and a stroke mimicker. We describe a case of 50 years old lady presented with acute onset headache, right hemi sensory tingling and bilateral papilloedema. Her blood test, MRI of brain and MRV was normal. CSF study revealed lymphocytic pleocytosis. The patient was discharged with full recovery. HaNDL syndrome is a diagnosis of exclusion. High degree of suspicion and characteristic clinical and laboratory findings are important to recognize. J Dhaka Medical College, Vol. 29, No.1, April, 2020, Page 89-91

‘Should have gone to …’: bilateral papilloedema with normal CSF pressure due to vestibular schwannoma

A 24-year-old woman presented with bilateral blurring of her distance vision and ‘dizzy spells’. She had no other neurological symptoms or medical history. She consulted an optometrist, and optical coherence tomography (OCT) was performed, which demonstrated papilloedema. She was referred to the local eye clinic for assessment and from there was referred for neurological assessment.Her initial investigations revealed no abnormalities, and brain imaging was reported to be normal. In the absence of an alternative diagnosis, idiopathic intracranial hypertension (IIH) was considered and a lumbar puncture was performed. This showed elevated protein but normal cerebrospinal fluid (CSF) pressure. MRI of the brain the next day revealed a large cerebellopontine lesion in keeping with vestibular schwannoma. She was referred to neurosurgery for operative management.This case highlights three interesting points: the aetiology of her papilloedema without raised intracranial pressure, the decision to perform a lumbar puncture in suspected IIH and community OCT as a clinical adjunct.

Cerebral Cystic Echinococcosis : A child with Giant Hydatid Cyst

Aim: Raising awareness of healthcare providers about headache and early morning vomiting in children. Background: Intracranial cystic hydatid disease (Echinococcosis) is a rare worldwide disease caused by encysted larvae of the dog tapeworm Echinococcus granulosa. Human is the intermediate host of this parasite and CNS involvement occurs in only 3%. Clinically, the cyst will cause seizure, increased intracranial pressure and/or focal neurological deficit. Surgical removal of the intact cyst by the Dowling technique is the treatment. A 4-year-old girl was referred to neurosurgery clinic with history of headache and early morning vomiting over the previous 2 months. Neurological examination revealed right hemiparesis and bilateral papilloedema. Emergency brain CT-scan revealed huge intracranial cystic lesion with midline shift. Emergency craniotomy was done and the cyst has been removed intact without complication and sent for histopathological examination. The patient has been discharged from the hospital with full recovery.

Spontaneous intracranial hypotension associated with pachymeningeal enhancement in a patient with systemic lupus erythematosus (SLE): an extremely rare presenting feature

A 16-years-old girl presented to our institution with history of severe bilateral headache for 5 days associated with vomiting. She also had fever for 2 months without any localising symptoms and skin lesions for 1 month. Examination revealed erythematous rash over bridge of nose and ear lobes, ulcer over hard palate and tenderness of small joints of both hands. Systemic examination was unremarkable except for bilateral papilloedema. Investigations revealed anaemia, leucopenia and elevated erythrocyte sedimentation rate. Cranial imaging revealed diffuse pachymeningeal enhancement with subdural effusion. Lumbar puncture revealed no abnormal findings in cerebrospinal fluid except low opening pressure. Antinuclear antibody was 4+ with anti-Sm antibody positive. She was diagnosed to have spontaneous intracranial hypotension associated with pachymeningeal enhancement secondary to systemic lupus erythematosus. She showed dramatic improvement with steroid and azathioprine therapy. She continues to be asymptomatic after 2 years of follow-up.