Orthostatic Hemifacial Spasm in Idiopathic Intracranial Hypertension.

BACKGROUND: Among the symptoms seen in idiopathic intracranial hypertension (IIH), hemifacial spasm (HFS) is rare. Orthostatic-induced HFS preceding lumbar puncture (LP) is previously unreported. We treated two patients with unusual IIH presentations. This case series reviews the few reports of HFS in IIH and proposes a mechanism for spasm occurrence. METHODS: Case 1: A woman in her mid-40s with previously controlled IIH developed daily headache, pulsatile tinnitus, right-sided trigeminal paresthesia, and right-sided HFS. The latter 2 symptoms occurred exclusively when moving from a sitting to a standing position. Imaging was unremarkable; opening pressure (OP) on LP was 46 cmH2O with normal cerebrospinal fluid (CSF) components. Case 2: A woman in her late 40s presented with severe daily headache, pulsatile tinnitus, and left-sided HFS following weight gain. Imaging was unremarkable; OP on LP was 32 cmH2O with normal CSF components. RESULTS: HFS episodes persisted following LP in both patients. Increasing and initiating acetazolamide, respectively, resolved all symptoms. CONCLUSIONS: Earlier suggested mechanisms of HFS are based on elevated intracranial pressure (ICP) shifting the facial nerve into proximity of a vascular structure. HFS appearing upon standing and continuing after LP, and thus a lower ICP, contradicts this. We propose a mechanism based on the degree of ICP change. This theory is grounded in the lack of intracranial compliance in IIH, wherein substantial pressure changes occur following small volume changes.

Blinding Optic Neuropathy Associated With Carboplatin Therapy: A Case Report and Literature Review.

Various forms of cancer and chemotherapeutics are associated with optic neuropathy. Cisplatin is a platinum analogue chemotherapeutic commonly associated with ocular toxicity among many other serious adverse effects. Carboplatin is a more chemically stable platinum analogue that is generally better tolerated with a comparatively favorable side effect profile. There are very few reports of carboplatin precipitating optic neuropathy. This case report describes a rare occurrence of carboplatin-induced blinding optic neuropathy. We treated a patient receiving carboplatin for neuroendocrine bladder cancer who developed rapidly progressive bilateral optic neuropathy over the course of three days. Upon evaluation at our clinic, his visual acuity had declined to light perception only and 20/60 in his left and right eye, respectively. Carboplatin therapy was immediately held and steroids were initiated. Despite the intervention, the patient's visual deficits have not improved at the one-year follow-up. Although the mechanism by which carboplatin causes ocular toxicity remains speculative, arterial ischemia appears to be the likely mechanism given the irreversible nature of visual decline. As demonstrated by our patient's course, irreversible vision loss despite high-dose steroid intervention necessitates expeditious recognition and management of this rare adverse effect. ​​​​​.

Chronic Inflammatory Demyelinating Polyneuropathy With Reversible Severe Cognitive Impairment and Gastrointestinal Dysfunction.

We treated a patient with an unusual case of reversible rapidly progressive cognitive impairment, gastrointestinal dysfunction, and generalized neuromyopathy in chronic inflammatory demyelinating polyneuropathy (CIDP) with optic neuropathy. A man in his 50s presented with a four-month history of rapidly progressive cognitive decline in addition to a six-month history of proximal greater than distal painful muscle weakness, wasting in all extremities, almost complete loss of deep tendon reflexes in his lower extremities, and slow progressive vision loss. Additionally, he had a 90-pound weight loss over the past two years with loss of appetite and ongoing chronic diarrhea. The exam showed muscle weakness and wasting with absent deep tendon reflexes. Initial Saint Louis University Mental Status (SLUMS) exam score was 16/30. Visual acuity was 20/25 with full extraocular movements; optical coherence tomography revealed superior arcuate bundle thinning bilaterally. Gastrointestinal workup proved nonrevealing. Serologic studies for vitamin deficiencies, heavy metals, and autoantibodies were negative. Whipple, Giardia lamblia, and Campylobacter jejuni stool testing were negative. Imaging studies were unremarkable. Nerve conduction studies showed demyelinating sensorimotor peripheral neuropathy. Muscle biopsy was indicative of denervation with scattered myopathic changes; no evidence of inflammatory myopathy nor glycogen or mitochondrial abnormalities was seen. Intravenous immunoglobulin treatment was begun. The patient was started at a dose of 0.75g/kg every three weeks. Following good but incomplete clinical improvement after the first treatment, his dose was increased to 1g/kg every three weeks. He improved remarkably after four months of infusions, scoring 30/30 on SLUMS with a full return of muscle strength and reflexes. Diarrhea remitted. Visual acuity and conduction delay remained unchanged. Symptom timing and dramatic response to immunoglobulins suggest a common immunological mechanism. In light of extensive differential investigations, unremarkable imaging and serology, and no other systemic disease processes, this case plausibly represents a potential new CIDP phenotypic variant.